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O2k-Publications: Fibroblast

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O2k-Publications: Fibroblast

O2k-Publications in the MiPMap

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NIH3T3 mouse embryonic fibroblast cells come from a cell line isolated and initiated in 1962 at the New York University School of Medicine Department of Pathology. 3T3 refers to the cell transfer and inoculation protocol for the line, and means 3-day transfer, inoculum 3 x 105 cells. Using this protocol, the immortal cell line begins to thrive and stabilize in cell culture after about 20-30 generations of in vitro growth. George Todaro and Howard Green, the scientists who first cultured this cell line, obtained the cells from desegregated NIH Swiss mouse embryo fibroblasts. The cell line has since become a standard fibroblast cell line.

Cell Culture Information

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 Was published in yearReferenceOrganismTissue;cell
MiPNet18.10 O2k-Specifications2024-03-26
Oroboros
O2k-specifications for respirometry and comprehensive OXPHOS analysis.
HumanFibroblast
Gaudo 2023 Free Radic Biol Med2023GaudĂł P, de TomĂĄs-Mateo E, Garrido-PĂ©rez N, Santana A, Ruiz-Pesini E, Montoya J, Bayona-Bafaluy P (2023) "ATAD3C regulates ATAD3A assembly and function in the mitochondrial membrane". https://doi.org/10.1016/j.freeradbiomed.2023.12.006HumanFibroblast
Robertson 2023 J Cell Sci2023Robertson GL, Riffle S, Patel M, Bodnya C, Marshall A, Beasley HK, Garza-Lopez E, Shao J, Vue Z, Hinton A, Stoll MS, de Wet S, Theart RP, Chakrabarty RP, Loos B, Chandel NS, Mears JA, Gama V (2023) DRP1 mutations associated with EMPF1 encephalopathy alter mitochondrial membrane potential and metabolic programs. https://doi.org/10.1242/jcs.260370HumanFibroblast
Curtabbi 2023 Redox Biol2023Curtabbi A, GuarĂĄs A, Cabrera-AlarcĂłn JL, Rivero M, Calvo E, Rosa-Moreno M, VĂĄzquez J, Medina M, EnrĂ­quez JA (2023) Regulation of respiratory complex I assembly by FMN cofactor targeting. https://doi.org/10.1016/j.redox.2023.103001MouseFibroblast
Kidney
Temelie 2023 Int J Mol Sci2023Temelie M, Talpur R, Dominguez-Prieto M, Dantas Silva A, Cenusa C, Craciun L, Savu DI, Moisoi N (2023) Impaired integrated stress response and mitochondrial integrity modulate genotoxic stress impact and lower the threshold for immune signalling. https://doi.org/10.3390/ijms24065891MouseFibroblast
Bassot 2023 Cell Rep2023Bassot A, Chen J, Takahashi-Yamashiro K, Yap MC, Gibhardt CS, Le GNT, Hario S, Nasu Y, Moore J, Gutiérrez T, Mina L, Mast H, Moses A, Bhat R, Ballanyi K, Lemieux H, Sitia R, Zito E, Bogeski I, Campbell RE, Simmen T (2023) The endoplasmic reticulum kinase PERK interacts with the oxidoreductase ERO1 to metabolically adapt mitochondria. Cell Rep 42:111899. https://doi.org/10.1016/j.celrep.2022.111899Human
Mouse
HEK
Fibroblast
Kamienieva 2023 Biochim Biophys Acta Mol Basis Dis2023Kamienieva I, CharzyƄska A, DuszyƄski J, MaliƄska D, Szczepanowska J (2023) In search for mitochondrial biomarkers of Parkinson's disease: Findings in parkin-mutant human fibroblasts. https://doi.org/10.1016/j.bbadis.2023.166787HumanFibroblast
Tian 2023 Redox Biol2023Tian G, Zhou J, Quan Y, Kong Q, Li J, Xin Y, Wu W, Tang X, Liu X (2023) Voltage-dependent anion channel 1 (VDAC1) overexpression alleviates cardiac fibroblast activation in cardiac fibrosis via regulating fatty acid metabolism. https://doi.org/10.1016/j.redox.2023.102907MouseHeart
Fibroblast
Lee 2022 Hum Mol Genet2022Lee RG, Balasubramaniam S, Stentenbach M, Kralj T, McCubbin T, Padman B, Smith J, Riley LG, Priyadarshi A, Peng L, Nuske MR, Webster R, Peacock K, Roberts P, Stark Z, Lemire G, Ito YA; Care4Rare Canada Consortium, Boycott KM, Geraghty MT, van Klinken JB, Ferdinandusse S, Zhou Y, Walsh R, Marcellin E, Thorburn DR, Rosciolli T, Fletcher J, Rackham O, Vaz FM, Reid GE, Filipovska A (2022) Deleterious variants in CRLS1 lead to cardiolipin deficiency and cause an autosomal recessive multi-system mitochondrial disease. https://doi.org/10.1093/hmg/ddac040HumanFibroblast
Zdrazilova 2022 PLOS ONE2022Zdrazilova L, Hansikova H, Gnaiger E (2022) Comparable respiratory activity in attached and suspended human fibroblasts. https://doi.org/10.1371/journal.pone.0264496HumanFibroblast
Adant 2022 Mol Metab2022Adant I, Bird M, Decru B, Windmolders P, Wallays M, de Witte P, Rymen D, Witters P, Vermeersch P, Cassiman D, GhesquiĂšre B (2022) Pyruvate and uridine rescue the metabolic profile of OXPHOS dysfunction. https://doi.org/10.1016/j.molmet.2022.101537HumanFibroblast
Kataura 2022 Dev Cell2022Kataura T, Sedlackova L, Otten EG, Kumari R, Shapira D, Scialo F, Stefanatos R, Ishikawa KI, Kelly G, Seranova E, Sun C, Maetzel D, Kenneth N, Trushin S, Zhang T, Trushina E, Bascom CC, Tasseff R, Isfort RJ, Oblong JE, Miwa S, Lazarou M, Jaenisch R, Imoto M, Saiki S, Papamichos-Chronakis M, Manjithaya R, Maddocks ODK, Sanz A, Sarkar S, Korolchuk VI (2022) Autophagy promotes cell survival by maintaining NAD levels. https://doi.org/10.1016/j.devcel.2022.10.008MouseFibroblast
Gschwind 2022 Ann Clin Transl Neurol2022Gschwind M, Garcia Segarra N, Schaller A, Bolognini R, Nuoffer JM, Hourez R, Deprez M, Lhermitte B, Maeder P, Tran C, Kuntzer T (2022) Early-onset leukoencephalomyelopathy due to a biallelic NDUFV1 variant in a mid-forties patient. https://doi.org/10.1002/acn3.51556HumanSkeletal muscle
Fibroblast
Schober 2022 Hum Mol Genet2022Schober FA, Tang JX, Sergeant K, Moedas MF, Zierz CM, Moore D, Smith C, Lewis D, Guha N, Hopton S, Falkous G, Lam A, Pyle A, Poulton J, Gorman GS, Taylor RW, Freyer C, Wredenberg A (2022) Pathogenic SLC25A26 variants impair SAH transport activity causing mitochondrial disease. https://doi.org/10.1093/hmg/ddac002Mouse
Drosophila
Fibroblast
Munoz-Pujol 2022 Brain Pathol2022Muñoz-Pujol G, Ortigoza-Escobar JD, Paredes-Fuentes AJ, Jou C, Ugarteburu O, Gort L, Yubero D, García-Cazorla A, O'Callaghan M, Campistol J, Muchart J, Yépez VA, Gusic M, Gagneur J, Prokisch H, Artuch R, Ribes A, Urreizti R, Tort F (2022) Leigh syndrome is the main clinical characteristic of PTCD3 deficiency. https://doi.org/10.1111/bpa.13134HumanFibroblast
Correia 2021 Hum Mutat2021Correia SP, Moedas MF, Naess K, Bruhn H, Maffezzini C, Calvo-Garrido J, Lesko N, Wibom R, Schober FA, Jemt A, Stranneheim H, Freyer C, Wedell A, Wredenberg A (2021) Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7. Hum Mutat 42:378-84.HumanFibroblast
Cretin 2021 EMBO Mol Med2021Cretin E, Lopes P, Vimont E, Tatsuta T, Langer T, Gazi A, Sachse M, Yu-Wai-Man P, Reynier P, Wai T (2021) High-throughput screening identifies suppressors of mitochondrial fragmentation in OPA1 fibroblasts. EMBO Mol Med 13:13579.MouseFibroblast
Friederich 2021 Mol Genet Metab2021Friederich MW, Geddes GC, Wortmann SB, Punnoose A, Wartchow E, Knight KM, Prokisch H, Creadon-Swindell G, Mayr JA, Van Hove JLK (2021) Pathogenic variants in MRPL44 cause infantile cardiomyopathy due to a mitochondrial translation defect. Mol Genet Metab 133:362-71.HumanFibroblast
Burska 2021 Biochim Biophys Acta Mol Basis Dis2021Burska D, Stiburek L, Krizova J, Vanisova M, Martinek V, Sladkova J, Zamecnik J, Honzik T, Zeman J, Hansikova H, Tesarova M (2021) Homozygous missense mutation in UQCRC2 associated with severe encephalomyopathy, mitochondrial complex III assembly defect and activation of mitochondrial protein quality control. Biochim Biophys Acta Mol Basis Dis 1867:166147.HumanFibroblast
Giordano 2021 Gene Ther2021Giordano L, Aneja MK, Sommer N, Alebrahimdehkordi N, Seraji A, Weissmann N, Rudolph C, Plank C, Jacobs HT, Szibor M (2021) Alternative oxidase encoded by sequence-optimized and chemically-modified RNA transfected into mammalian cells is catalytically active. https://doi.org/10.1038/s41434-021-00235-zMouseFibroblast
Gnaiger 2021 MitoFit BCA2021Gnaiger E (2021) Bioenergetic cluster analysis – mitochondrial respiratory control in human fibroblasts. MitoFit Preprints 2021.08. https://doi.org/10.26124/mitofit:2021-0008HumanFibroblast
Brito 2021 BMC Cancer2021Brito A, Merle C, Lagarde P, Faustin B, Devin A, Lartigue L, Chibon F (2021) Cell fusion enhances energy metabolism of mesenchymal tumor hybrid cells to sustain their proliferation and invasion. BMC Cancer 21:863.HumanFibroblast
Baldini 2021 Life Sci2021Baldini F, Fabbri R, Eberhagen C, Voci A, Portincasa P, Zischka H, Vergani L (2021) Adipocyte hypertrophy parallels alterations of mitochondrial status in a cell model for adipose tissue dysfunction in obesity. Life Sci 265:118812.MouseFibroblast
Hesse 2021 FASEB J2021Hesse J, Groterath W, Owenier C, Steinhausen J, Ding Z, Steckel B, Czekelius C, Alter C, Marzoq A, Schrader J (2021) Normoxic induction of HIF-1α by adenosine-A2B R signaling in epicardial stromal cells formed after myocardial infarction. FASEB J 35:21517.RatHeart
Fibroblast
Kripnerova 2021 J Clin Med2021KripnerovĂĄ M, Parmar HS, Ć ĂĄna J, KopkovĂĄ A, RadovĂĄ L, Sopper S, Biernacki K, Jedlička J, KohoutovĂĄ M, KuncovĂĄ J, Peychl J, Rudolf E, Červinka M, Houdek Z, Dvoƙák P, HoufkovĂĄ K, PeĆĄta M, TĆŻma Z, DolejĆĄovĂĄ M, TichĂĄnek F, BabuĆĄka V, Leba M, SlabĂœ O, Hatina J (2021) Complex interplay of genes underlies invasiveness in fibrosarcoma progression model. J Clin Med 10:2297.MouseFibroblast
Cassereau 2020 Exp Neurol2020Cassereau J, Chevrollier A, Codron P, Goizet C, Gueguen N, Verny C, Reynier P, Bonneau D, Lenaers G, Procaccio V (2020) Oxidative stress contributes differentially to the pathophysiology of Charcot-Marie-Tooth disease type 2K. Exp Neurol 323:113069.HumanFibroblast
Gutierrez 2020 Sci Signal2020Gutiérrez T, Qi H, Yap MC, Tahbaz N, Milburn LA, Lucchinetti E, Lou PH, Zaugg M, LaPointe PG, Mercier P, Overduin M, Bischof H, Burgstaller S, Malli R, Ballanyi K, Shuai J, Simmen T (2020) The ER chaperone calnexin controls mitochondrial positioning and respiration . Sci Signal 13:eaax6660.MouseFibroblast
Xia 2020 Biomed Res Int2020Xia W, Wang Q, Lu Y, Hu Y, Zhang X, Zhang J, Liu D, Song J, Zhu Z, Liu D, Zhang H (2020) Transient receptor potential channel canonical type 3 deficiency antagonizes myofibroblast transdifferentiation in vivo. Biomed Res Int 2020:1202189.HumanFibroblast
Kawamura 2021 Mol Ther Nucleic Acids2020Kawamura E, Maruyama M, Abe J, Sudo A, Takeda A, Takada S, Yokota T, Kinugawa S, Harashima H, Yamada Y (2021) Validation of gene therapy for mutant mitochondria by delivering mitochondrial RNA using a MITO-porter. Mol Ther Nucleic Acids 20:687-98.HumanFibroblast
Alahmad 2020 EMBO Mol Med2020Alahmad A, Nasca A, Heidler J, Thompson K, OlĂĄhovĂĄ M, Legati A, Lamantea E, Meisterknecht J, Spagnolo M, He L, Alameer S, Hakami F, Almehdar A, Ardissone A, Alston CL, McFarland R, Wittig I, Ghezzi D, Taylor RW (2020) Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I. EMBO Mol Med 12:e12619.HumanFibroblast
Juarez-Flores 2020 Antioxidants (Basel)2020Juarez-Flores DL, Ezquerra M, Gonzalez-Casacuberta I, Ormazabal A, Moren C, Tolosa E, Fucho R, Guitart-Mampel M, Casado M, Valldeoriola F, de la Torre-Lara J, Munoz E, Tobias E, Compta Y, Garcia-Garcia FJ, Garcia-Ruiz C, Fernandez-Checa JC, Marti MJ, Grau JM, Cardellach F, Artuch R, Fernandez-Santiago R, Garrabou G (2020) Disrupted mitochondrial and metabolic plasticity underlie comorbidity between age-related and degenerative disorders as Parkinson disease and type 2 diabetes mellitus. Antioxidants (Basel) 9:1063.HumanFibroblast
Pelaez Coyotl 2020 Pharmaceutics2020Pelåez Coyotl EA, Barrios Palacios J, Muciño G, Moreno-Blas D, Costas M, Montiel Montes T, Diener C, Uribe-Carvajal S, Massieu L, Castro-Obregón S, Espinosa OR, Mata Espinosa D, Barrios-Payan J, León Contreras JC, Corzo G, Hernåndez-Pando R, Del Rio G (2020) Antimicrobial peptide against Mycobacterium tuberculosis that activates autophagy is an effective treatment for tuberculosis. Pharmaceutics 12:E1071.MouseFibroblast
Ugarteburu 2020 J Clin Med2020Ugarteburu O, Sånchez-Vilés M, Ramos J, Barcos-Rodríguez T, Garrabou G, García-Villoria J, Ribes A, Tort F (2020) Physiopathological bases of the disease caused by HACE1 mutations: alterations in autophagy, mitophagy and oxidative stress response. J Clin Med 9:E913.HumanFibroblast
Alston 2020 Am J Hum Genet2020Alston CL, Veling MT, Heidler J, Taylor LS, Alaimo JT, Sung AY, He L, Hopton S, Broomfield A, Pavaine J, Diaz J, Leon E, Wolf P, McFarland R, Prokisch H, Wortmann SB, Bonnen PE, Wittig I, Pagliarini DJ, Taylor RW (2020) Pathogenic bi-allelic mutations in NDUFAF8 cause Leigh syndrome with an isolated complex I deficiency. Am J Hum Genet 106:92-101.HumanFibroblast
Gnaiger 2020 BEC MitoPathways2020Gnaiger E (2020) Mitochondrial pathways and respiratory control. An introduction to OXPHOS analysis. 5th ed. Bioenerg Commun 2020.2. https://doi.org/10.26124/bec:2020-0002Human
Mouse
Heart
Skeletal muscle
Fibroblast
Catalan-Garcia 2020 J Clin Med2020CatalĂĄn-GarcĂ­a M, GarcĂ­a-GarcĂ­a FJ, Moreno-Lozano PJ, Alcarraz-VizĂĄn G, Tort-Merino A, Milisenda JC, CantĂł-Santos J, Barcos-RodrĂ­guez T, Cardellach F, LladĂł A, Novials A, Garrabou G, Grau-Junyent JM (2020) Mitochondrial dysfunction: A common hallmark underlying comorbidity between sIBM and other degenerative and age-related diseases. J Clin Med 9:E1446.HumanFibroblast
Cornelissen 2020 Hum Mol Genet2020Cornelissen T, Spinazzi M, Martin S, Imberechts D, Vangheluwe P, Bird M, De Strooper B, Vandenberghe W (2020) CHCHD2 harboring the Parkinson's disease-linked T61I mutation precipitates inside mitochondria and induces precipitation of wild-type CHCHD2. Hum Mol Genet 29:1096-106.HumanFibroblast
Robledo-Cadena 2020 Pharmaceuticals (Basel)2020Robledo-Cadena DX, Gallardo-PĂ©rez JC, DĂĄvila-Borja V, Pacheco-VelĂĄzquez SC, Belmont-DĂ­az JA, Ralph SJ, Blanco-Carpintero BA, Moreno-SĂĄnchez R, RodrĂ­guez-EnrĂ­quez S (2020) Non-steroidal anti-inflammatory drugs increase cisplatin, paclitaxel, and doxorubicin efficacy against human cervix cancer cells. Pharmaceuticals (Basel) 13:463.HumanEndothelial;epithelial;mesothelial cell
HeLa
Fibroblast
Beninca 2020 J Med Genet2020Beninca C, Zanette V, Brischigliaro M, Johnson M, Reyes A, Valle DAD, Robinson AJ, Degiorgi A, Yeates A, Telles BA, Prudent J, Baruffini E, Santos ML, de Souza RL, Fernandez-Vizarra E, Whitworth A, Zeviani M (2020) Mutation in the MICOS subunit gene APOO (MIC26) associated with an X-linked recessive mitochondrial myopathy, lactic acidosis, cognitive impairment and autistic features. J Med Genet 58:155-67.HumanFibroblast
Zhao 2019 Basic Res Cardiol2019Zhao J, Gao JL, Zhu JX, Zhu HB, Peng X, Jiang M, Fu Y, Xu J, Mao XH, Hu N, Ma MH, Dong DL (2019) The different response of cardiomyocytes and cardiac fibroblasts to mitochondria inhibition and the underlying role of STAT3. Basic Res Cardiol 114:12.RatHeart
Fibroblast
Friederich 2019 Mol Genet Metab2019Friederich MW, Perez FA, Knight KM, Van Hove RA, Yang SP, Saneto RP, Van Hove JLK (2019) Pathogenic variants in NUBPL result in failure to assemble the matrix arm of complex I and cause a complex leukoencephalopathy with thalamic involvement. Mol Genet Metab 129:236-42.HumanFibroblast
Desquiret-Dumas 2019 Biochim Biophys Acta Mol Basis Dis2019Desquiret-Dumas V, Leman G, Wetterwald C, Chupin S, Lebert A, Khiati S, Le Mao M, Geffroy G, Kane MS, Chevrollier A, Goudenege D, Gadras C, Tessier L, Barth M, Leruez S, Amati-Bonneau P, Henrion D, Bonneau D, Procaccio V, Reynier P, Lenaers G, Gueguen N (2019) Warburg-like effect is a hallmark of complex I assembly defects. Biochim Biophys Acta Mol Basis Dis 1865:2475-89.HumanFibroblast
Wolf 2019 Cells2019Wolf C, Zimmermann R, Thaher O, Bueno D, WĂŒllner V, SchĂ€fer MKE, Albrecht P, Methner A (2019) The Charcot-Marie tooth disease mutation R94Q in MFN2 decreases ATP production but increases mitochondrial respiration under conditions of mild oxidative stress. Cells 8:E1289.MouseFibroblast
Silva Ramos 2019 PLoS Genet2019Silva Ramos E, Motori E, BrĂŒser C, KĂŒhl I, Yeroslaviz A, Ruzzenente B, Kauppila JHK, Busch JD, Hultenby K, Habermann BH, Jakobs S, Larsson NG, Mourier A (2019) Mitochondrial fusion is required for regulation of mitochondrial DNA replication. PLoS Genet 15:e1008085.MouseHeart
Fibroblast
Krylova 2019 Mitochondrion2019Krylova TD, Sheremet NL, Tabakov VY, Lyamzaev KG, Itkis YS, Tsygankova PG, Andreeva NA, Shmelkova MS, Nevinitsyna TA, Kadyshev VV, Zakharova EY (2019) Three rare pathogenic mtDNA substitutions in LHON patients with low heteroplasmy. Mitochondrion 50:139-144.HumanFibroblast
Germain 2019 Int J Med Sci2019Germain N, Dessein AF, Vienne JC, Dobbelaere D, Mention K, Joncquel M, Dekiouk S, Laine W, Kluza J, Marchetti P (2019) First-line screening of OXPHOS deficiencies using microscale oxygraphy in human skin fibroblasts: A preliminary study. Int J Med Sci 16:931-38.HumanFibroblast
Richter 2019 Life Sci Alliance2019Richter U, Ng KY, Suomi F, Marttinen P, Turunen T, Jackson C, Suomalainen A, Vihinen H, Jokitalo E, Nyman TA, Isokallio MA, Stewart JB, Mancini C, Brusco A, Seneca S, LombĂšs A, Taylor RW, Battersby BJ (2019) Mitochondrial stress response triggered by defects in protein synthesis quality control. Life Sci Alliance 2:e201800219.HumanFibroblast
Gustafson 2019 PLoS One2019Gustafson MA, McCormick EM, Perera L, Longley MJ, Bai R, Kong J, Dulik M, Shen L, Goldstein AC, McCormack SE, Laskin BL, Leroy BP, Ortiz-Gonzalez XR, Ellington MG, Copeland WC, Falk MJ (2019) Mitochondrial single-stranded DNA binding protein novel de novo SSBP1 mutation in a child with single large-scale mtDNA deletion (SLSMD) clinically manifesting as Pearson, Kearns-Sayre, and Leigh syndromes. PLoS One 14:e0221829.HumanFibroblast
Lymphocyte
Itkis 2019 Mitochondrion2019Itkis Y, Krylova T, Pechatnikova NL, De Grassi A, Tabakov VY, Pierri CL, Aleshin V, Boyko A, Bunik VI, Zakharova EY (2019) A novel variant m.641A>T in the mitochondrial MT-TF gene is associated with epileptic encephalopathy in adolescent. Mitochondrion 47:10-17.HumanFibroblast
Wang H 2019 J Biol Chem2019Wang H, Lu J, Kulkarni S, Zhang W, Gorka JE, Mandel JA, Goetzman ES, Prochownik EV (2019) Metabolic and oncogenic adaptations to pyruvate dehydrogenase inactivation in fibroblasts. J Biol Chem 294:5466-86.RatFibroblast
Veyrat-Durebex 2019 Mol Neurobiol2019Veyrat-Durebex C, Bris C, Codron P, Bocca C, Chupin S, Corcia P, Vourc'h P, Hergesheimer R, Cassereau J, Funalot B, Andres CR, Lenaers G, Couratier P, Reynier P, Blasco H (2019) Metabo-lipidomics of fibroblasts and mitochondrial-endoplasmic reticulum extracts from ALS patients shows alterations in purine, pyrimidine, energetic, and phospholipid metabolisms. Mol Neurobiol 56:5780-91.HumanFibroblast
Gaudo 2019 Neurogenetics2019GaudĂł P, Emperador S, Garrido-PĂ©rez N, Ruiz-Pesini E, Yubero D, GarcĂ­a-Cazorla A, Artuch R, Montoya J, Bayona-Bafaluy MP (2019) Infectious stress triggers a POLG-related mitochondrial disease. Neurogenetics 21:19-27.HumanFibroblast
Barca 2018 Hum Mol Genet2018Barca E, Ganetzky RD, Potluri P, Juanola-Falgarona M, Gai X, Li D, Jalas C, Hirsch Y, Emmanuele V, Tadesse S, Ziosi M, Akman HO, Chung WK, Tanji K, McCormick E, Place E, Consugar M, Pierce EA, Hakonarson H, Wallace DC, Hirano M, Falk MJ (2018) USMG5 Ashkenazi Jewish founder mutation impairs mitochondrial complex V dimerization and ATP synthesis. Hum Mol Genet 27:3305-12.HumanFibroblast
Harmuth 2018 Front Mol Neurosci2018Harmuth T, Prell-Schicker C, Weber JJ, Gellerich F, Funke C, Drießen S, Magg JCD, Krebiehl G, Wolburg H, Hayer SN, Hauser S, KrĂŒger R, Schöls L, Riess O, HĂŒbener-Schmid J (2018) Mitochondrial morphology, function and homeostasis are impaired by expression of an N-terminal calpain cleavage fragment of Ataxin-3. Front Mol Neurosci 11:368.MouseFibroblast
Szelechowski 2018 Sci Rep2018Szelechowski M, Amoedo N, Obre E, LĂ©ger C, Allard L, Bonneu M, Claverol S, Lacombe D, Oliet S, Chevallier S, Le Masson G, Rossignol R (2018) Metabolic reprogramming in amyotrophic lateral sclerosis. Sci Rep 8:3953.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Reyes 2018 EMBO Mol Med2018Reyes A, Melchionda L, Burlina A, Robinson AJ, Ghezzi D, Zeviani M (2018) Mutations in TIMM50 compromise cell survival in OxPhos-dependent metabolic conditions. EMBO Mol Med 10:e8698.HumanFibroblast
Monzio Compagnoni 2018 Biochim Biophys Acta Mol Basis Dis2018Monzio Compagnoni G, Kleiner G, Bordoni A, Fortunato F, Ronchi D, Salani S, Guida M, Corti C, Pichler I, Bergamini C, Fato R, Pellecchia MT, Vallelunga A, Del Sorbo F, Elia A, Reale C, Garavaglia B, Mora G, Albanese A, Cogiamanian F, Ardolino G, Bresolin N, Corti S, Comi GP, Quinzii CM, Di Fonzo A (2018) Mitochondrial dysfunction in fibroblasts of multiple system atrophy. Biochim Biophys Acta Mol Basis Dis 1864:3588-97.HumanFibroblast
Walczak 2018 FASEB J2018Walczak J, Dębska-Vielhaber G, Vielhaber S, SzymaƄski J, CharzyƄska A, DuszyƄski J, Szczepanowska J (2018) Distinction of sporadic and familial forms of ALS based on mitochondrial characteristics. FASEB J 33:4388-403.HumanFibroblast
Sant'Anna-Silva 2018 Front Oncol2018Sant'Anna-Silva ACB, Santos GC, Campos SPC, Oliveira Gomes AM, PĂ©rez-Valencia JA, Rumjanek FD (2018) Metabolic profile of oral squamous carcinoma cell lines relies on a higher demand of lipid metabolism in metastatic cells. Front Oncol 8:13.HumanOther cell lines
Fibroblast
Chen 2018 Hum Mol Genet2018Chen A, Tiosano D, Guran T, Baris HN, Bayram Y, Mory A, Shapiro-Kulnane L, Hodges CA, Coban Akdemir Z, Turan S, Jhangiani SN, van den Akker F, Hoppel CL, Salz HK, Lupski JR, Buchner DA (2018) Mutations in the mitochondrial ribosomal protein MRPS22 lead to primary ovarian insufficiency. Hum Mol Genet 27:1913-26.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Giordano 2018 Am J Respir Cell Mol Biol2018Giordano L, Farnham A, Dhandapani PK, Salminen L, Bhaskaran J, Voswinckel R, Rauschkolb P, Scheibe S, Sommer N, Beisswenger C, Weissmann N, Braun T, Jacobs HT, Bals R, Herr C, Szibor M (2018) Alternative oxidase attenuates cigarette smoke-induced lung dysfunction and tissue damage. Am J Respir Cell Mol Biol 60:515-22.MouseFibroblast
Nacarelli 2018 Geroscience2018Nacarelli T, Azar A, Altinok O, Orynbayeva Z, Sell C (2018) Rapamycin increases oxidative metabolism and enhances metabolic flexibility in human cardiac fibroblasts. Geroscience 40:243-56.HumanHeart
Fibroblast
Boutoual 2018 Scientific Reports2018Boutoual, R., Meseguer, S., Villarroya, M., Martin-Hernandez, E., Errami, M., Martin, M. A., Casado, M., and Armengod, M. E. (2018) Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. Scientific reports 8, 1163HumanOther cell lines
Fibroblast
Alston 2018 Am J Hum Genet2018Alston CL, Heidler J, Dibley MG, Kremer LS, Taylor LS, Fratter C, French CE, Glasgow RIC, Feichtinger RG, Delon I, Pagnamenta AT, Dolling H, Lemonde H, Aiton N, BjĂžrnstad A, Henneke L, GĂ€rtner J, Thiele H, Tauchmannova K, Quaghebeur G, Houstek J, Sperl W, Raymond FL, Prokisch H, Mayr JA, McFarland R, Poulton J, Ryan MT, Wittig I, Henneke M, Taylor RW (2018) Bi-allelic mutations in NDUFA6 establish its role in early-onset isolated mitochondrial complex I deficiency. Am J Hum Genet 103:592-601.HumanFibroblast
Liu 2018 J Periodontol2018Liu J, Zeng J, Wang X, Zheng M, Luan Q (2018) P53 mediates lipopolysaccharide-induced inflammation in human gingival fibroblasts. J Periodontol 89:1142-51.HumanFibroblast
Temelie 2018 Oxid Med Cell Longev2018Temelie M, Savu DI, Moisoi N (2018) Intracellular and intercellular signalling mechanisms following DNA damage are modulated by PINK1. Oxid Med Cell Longev 1391387.Human
Mouse
Nervous system
Other cell lines
Fibroblast
Isohanni 2018 Neurogenetics2018Isohanni P, Carroll CJ, Jackson CB, Pohjanpelto M, Lönnqvist T, Suomalainen A (2018) Defective mitochondrial ATPase due to rare mtDNA m.8969G>A mutation-causing lactic acidosis, intellectual disability, and poor growth. Neurogenetics 19:49-53.HumanSkeletal muscle
Blood cells
Fibroblast
Lee 2018 Neurol Genet2018Lee RG, Sedghi M, Salari M, Shearwood AJ, Stentenbach M, Kariminejad A, Goullee H, Rackham O, Laing NG, Tajsharghi H, Filipovska A (2018) Early-onset Parkinson disease caused by a mutation in CHCHD2 and mitochondrial dysfunction. Neurol Genet 4:e276.HumanFibroblast
Dejmek 2018 Physiol Res2018Dejmek J, Kohoutová M, Kripnerová M, Čedíková M, TƯma Z, Babuơka V, Bolek L, Kuncová J (2018) Repeated exposure to hyperbaric hyperoxia affects mitochondrial functions of the lung fibroblasts. Physiol Res 67(Suppl 4):S633-S643.HumanLung;gill
Fibroblast
Juarez-Flores 2018 J Transl Med2018Juårez-Flores DL, Gonzålez-Casacuberta I, Ezquerra M, Bañó M, Carmona-Pontaque F, Catalån-García M, Guitart-Mampel M, Rivero JJ, Tobias E, Milisenda JC, Tolosa E, Marti MJ, Fernåndez-Santiago R, Cardellach F, Morén C, Garrabou G (2018) Exhaustion of mitochondrial and autophagic reserve may contribute to the development of LRRK2G2019S -Parkinson's disease. J Transl Med 16:160.HumanFibroblast
Friederich 2018 Nat Commun2018Friederich MW, Timal S, Powell CA, Dallabona C, Kurolap A, Palacios-Zambrano S, Bratkovic D, Derks TGJ, Bick D, Bouman K, Chatfield KC, Damouny-Naoum N, Dishop MK, Falik-Zaccai TC, Fares F, Fedida A, Ferrero I, Gallagher RC, Garesse R, Gilberti M, GonzĂĄlez C, Gowan K, Habib C, Halligan RK, Kalfon L, Knight K, Lefeber D, Mamblona L, Mandel H, Mory A, Ottoson J, Paperna T, Pruijn GJM, Rebelo-Guiomar PF, Saada A, Sainz B Jr, Salvemini H, Schoots MH, Smeitink JA, Szukszto MJ, Ter Horst HJ, van den Brandt F, van Spronsen FJ, Veltman JA, Wartchow E, Wintjes LT, Zohar Y, FernĂĄndez-Moreno MA, Baris HN, Donnini C, Minczuk M, Rodenburg RJ, Van Hove JLK (2018) Pathogenic variants in glutamyl-tRNAGln amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder. Nat Commun 9:4065.Human
Fungi
Fibroblast
Boutoual 2018 Sci Rep2018Boutoual R, Meseguer S, Villarroya M, MartĂ­n-HernĂĄndez E, Errami M, MartĂ­n MA, Casado M, Armengod ME (2018) Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARÎł-UCP2-AMPK axis. Sci Rep 8:1163.HumanFibroblast
Angebault 2018 Sci Signal2018Angebault C, Fauconnier J, Patergnani S, Rieusset J, Danese A, Affortit CA, Jagodzinska J, MĂ©gy C, Quiles M, Cazevieille C, Korchagina J, Bonnet-Wersinger D, Milea D, Hamel C, Pinton P, Thiry M, Lacampagne A, Delprat B, Delettre C (2018) ER-mitochondria cross-talk is regulated by the Ca2+ sensor NCS1 and is impaired in Wolfram syndrome. Sci Signal 11:eaaq1380.HumanFibroblast
Gerber 2017 Brain2017Gerber S, Charif M, Chevrollier A, Chaumette T, Angebault C, Kane MS, Paris A, Alban J, Quiles M, Delettre C, Bonneau D, Procaccio V, Amati-Bonneau P, Reynier P, Leruez S, Calmon R, Boddaert N, Funalot B, Rio M, Bouccara D, Meunier I, Sesaki H, Kaplan J, Hamel CP, Rozet JM, Lenaers G (2017) Mutations in DNM1L, as in OPA1, result indominant optic atrophy despite opposite effects on mitochondrial fusion and fission. Brain 140:2586-96.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Jang 2017 Biol Open2017Jang DH, Seeger SC, Grady ME, Shofer FC, Eckmann DM (2017) Mitochondrial dynamics and respiration within cells with increased open pore cytoskeletal meshes. Biol Open 6:1831-9.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Boutant 2017 EMBO J2017Boutant M, Kulkarni SS, Joffraud M, Ratajczak J, Valera-Alberni M, Combe R, Zorzano A, CantĂł C (2017) Mfn2 is critical for brown adipose tissue thermogenic function. EMBO J 36:1543-58.MouseFat
Fibroblast
Beresewicz 2017 PLOS ONE2017Beręsewicz M, BoratyƄska-JasiƄska A, Charzewski Ɓ, Kawalec M, KabziƄska D, KochaƄski A, Krzyƛko KA, ZabƂocka B (2017) The effect of a novel c.820C>T (Arg274Trp) mutation in the mitofusin 2 gene on fibroblast metabolism and clinical manifestation in a patient. PLOS ONE 12:e0169999.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Elmer 2017 US Patent2017Elmer E, Hansson MJ, Ehinger JK, Piel S, Moss S (2017) Succinate prodrugs for use in the treatment of lactic acidosis or drug-induced side-effects due to complex I-related impairment of mitochondrial oxidative phosphorylation. US Patent US20170100359 A1.HumanHeart
Blood cells
Fibroblast
Macrophage-derived
Platelet
Zhou 2017 Biochim Biophys Acta2017Zhou L, Wang W, Hoppel C, Liu J, Zhu X (2017) Parkinson's disease-associated pathogenic VPS35 mutation causes complex I deficits. Biochim Biophys Acta 1863:2791-5.HumanFibroblast
Wetzel 2017 Thesis2017Wetzel MD (2017) Mechanism of metformin action in dermal fibroblasts. PhD Thesis 125.HumanFibroblast
Porras 2017 Stem Cells2017Porras DP, Abbaszadeh M, Bhattacharya D, D'Souza NC, Edjiu NR, Perry CG, ScimĂš A (2017) p107 determines a metabolic checkpoint required for adipocyte lineage fates. Stem Cells 35:1378-91.MouseFat
Fibroblast
Scrima 2017 PLOS ONE2017Scrima R, Menga M, Pacelli C, Agriesti F, Cela O, Piccoli C, Cotoia A, De Gregorio A, Gefter JV, Cinnella G, Capitanio N (2017) Para-hydroxyphenylpyruvate inhibits the pro-inflammatory stimulation of macrophage preventing LPS-mediated nitro-oxidative unbalance and immunometabolic shift. PLOS ONE 12:e0188683.Human
Mouse
Endothelial;epithelial;mesothelial cell
Blood cells
Fibroblast
Macrophage-derived
Pfeiffer 2017 Free Radic Biol Med2017Pfeiffer A, Schneider J, Bueno D, Dolga A, Voss TD, Lewerenz J, WĂŒllner V, Methner A (2017) Bcl-xL knockout attenuates mitochondrial respiration and causes oxidative stress that is compensated by pentose phosphate pathway activity. Free Radic Biol Med 112:350-9.MouseFibroblast
Victor 2017 Thesis2017Victor L (2017) Investigating the possible cytoprotective effects of melatonin isomer against simulated ischemic injury. Master's Thesis 76.RatFibroblast
Uzhachenko 2017 Aging (Albany NY)2017Uzhachenko R, Boyd K, Olivares-Villagomez D, Zhu Y, Goodwin JS, Rana T, Shanker A, Tan WJ, Bondar T, Medzhitov R, Ivanova AV (2017) Mitochondrial protein Fus1/Tusc2 in premature aging and age-related pathologies: critical roles of calcium and energy homeostasis. Aging (Albany NY) 9:627-49.MouseKidney
Endothelial;epithelial;mesothelial cell
Fibroblast
Pravenec 2017 Physiol Res2017Pravenec M1, ZĂ­dek V, Landa V, Mlejnek P, Ć ilhavĂœ J, Ć imĂĄkovĂĄ M, TrnovskĂĄ J, Ć kop V, MarkovĂĄ I, MalĂ­nskĂĄ H, HĂŒttl M, KazdovĂĄ L, BardovĂĄ K, TauchmannovĂĄ K, VrbackĂœ M, NĆŻskovĂĄ H, Mráček T, KopeckĂœ J, HouĆĄtěk J (2017) Mutant Wars2 gene in spontaneously hypertensive rats impairs brown adipose tissue function and predisposes to visceral obesity. Physiol Res 66:917-24.RatEndothelial;epithelial;mesothelial cell
Fibroblast
Uppala 2017 Biochem Biophys Res Commun2017Uppala R, Dudiak B, Beck ME, Bharathi SS, Zhang Y, Stolz DB, Goetzman ES (2017) Aspirin increases mitochondrial fatty acid oxidation. Biochem Biophys Res Commun 482:346-51.Human
Mouse
HEK
Fibroblast
Ruiz 2017 Brain Pathol2017Ruiz M, Bégou M, Launay N, Ranea-Robles P, Bianchi P, López-Erauskin J, Morató L, Guilera C, Petit B, Vaurs-Barriere C, Guéret-Gonthier C, Bonnet-Dupeyron MN, Fourcade S, Auwerx J, Boespflug-Tanguy O, Pujol A (2017) Oxidative stress and mitochondrial dynamics malfunction are linked in Pelizaeus-Merzbacher disease. Brain Pathol 28:611-30.Human
Mouse
Nervous system
Fibroblast
Thaher 2017 Neurochem Int2017Thaher O, Wolf C, Dey PN, Pouya A, WĂŒllner V, Tenzer S, Methner A (2017) The thiol switch C684 in Mitofusin-2 mediates redox-induced alterations of mitochondrial shape and respiration. Neurochem Int 117:167-73.MouseFibroblast
Friederich 2017 Hum Mol Genet2017Friederich MW, Erdogan AJ, Coughlin CR 2nd, Elos MT, Jiang H, O'Rourke CP, Lovell MA, Wartchow E, Gowan K, Chatfield KC, Chick WS, Spector EB, Van Hove JLK, Riemer J (2017) Mutations in the accessory subunit NDUFB10 result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly. Hum Mol Genet 26:702-16.HumanFibroblast
Kane 2017 J Cell Mol Med2017Kane MS, Alban J, Desquiret-Dumas V, Gueguen N, Ishak L, Ferre M, Amati-Bonneau P, Procaccio V, Bonneau D, Lenaers G, Reynier P, Chevrollier A (2017) Autophagy controls the pathogenicity of OPA1 mutations in dominant optic atrophy. J Cell Mol Med 21:2284-97.HumanFibroblast
Krylova 2017 Biomed Khim2017Krylova TD, Tsygankova PG, Itkis YS, Sheremet NL, Nevinitsyna TA, Mikhaylova SV, Zakharova EY (2017) High resolution respirometry in diagnostic of mitochondrial disorders caused by mitochondrial complex I deficiency. Biomed Khim 63:327-33.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Rohde 2017 Cell Death Differ2017Rohde K, Kleinesudeik L, Roesler S, Löwe O, Heidler J, Schröder K, Wittig I, Dröse S, Fulda S (2017) A Bak-dependent mitochondrial amplification step contributes to Smac mimetic/glucocorticoid-induced necroptosis. Cell Death Differ 24:83-97.MouseFibroblast
Theisen 2017 Am J Med Genet A2017Theisen BE, Rumyantseva A, Cohen JS, Alcaraz WA, Shinde DN, Tang S, Srivastava S, Pevsner J, Trifunovic A, Fatemi A (2017) Deficiency of WARS2, encoding mitochondrial tryptophanyl tRNA synthetase, causes severe infantile onset leukoencephalopathy. Am J Med Genet A 173:2505-10.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Beresewicz 2017 PLoS One2017Beresewicz M, Boratynska-Jasinska A, Charzewski L, Kawalec M, Kabzinska D, Kochanski A, Krzysko KA, Zablocka B (2017) The effect of a novel c.820C>T (Arg274Trp) mutation in the mitofusin 2 gene on fibroblast metabolism and clinical manifestation in a patient. PLoS One 12:0169999.HumanFibroblast
Endothelial;epithelial;mesothelial cell
Chubanov 2016 Elife2016Chubanov V, Ferioli S, Wisnowsky A, Simmons DG, Leitzinger C, Einer C, Jonas W, Shymkiv Y, Bartsch H, Braun A, Akdogan B, Mittermeier L, Sytik L, Torben F, Jurinovic V, van der Vorst EP, Weber C, Yildirim ÖA, Sotlar K, SchĂŒrmann A, Zierler S, Zischka H, Ryazanov AG, Gudermann T (2016) Epithelial magnesium transport by TRPM6 is essential for prenatal development and adult survival. Elife 5. pii: e20914.HumanFibroblast
Other cell lines
Hartmannova 2016 Hum Mol Genet2016HartmannovĂĄ H, PiherovĂĄ L, TauchmannovĂĄ K, Kidd K, Acott PD, Crocker JF, Oussedik Y, Mallet M, HodaƈovĂĄ K, StrĂĄneckĂœ V, PƙistoupilovĂĄ A, BareĆĄovĂĄ V, JedličkovĂĄ I, ĆœivnĂĄ M, SovovĂĄ J, HĆŻlkovĂĄ H, Robins V, VrbackĂœ M, Pecina P, KaplanovĂĄ V, HouĆĄtěk J, Mráček T, Thibeault Y, Bleyer AJ, Kmoch S (2016) Acadian variant of Fanconi syndrome is caused by mitochondrial respiratory chain complex I deficiency due to a non-coding mutation in complex I assembly factor NDUFAF6. Hum Mol Genet 25:4062-79.HumanFibroblast
Wos 2016 Arch Biochem Biophys2016Woƛ M, Szczepanowska J, PikuƂa S, Tylki-SzymaƄska A, ZabƂocki K, Bandorowicz-PikuƂa J (2016) Mitochondrial dysfunction in fibroblasts derived from patients with Niemann-Pick type C disease. Arch Biochem Biophys 593:50-9.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Zelenka 2016 Oxid Med Cell Longev2016Zelenka J, Dvoƙák A, Alán L, Zadinová M, Haluzík M, Vítek L (2016) Hyperbilirubinemia protects against aging-associated inflammation and metabolic deterioration. Oxid Med Cell Longev ID 6190609:10p.RatLiver
Kidney
Fibroblast
Silva Ramos 2016 Biochim Biophys Acta2016Silva Ramos E, Larsson NG, Mourier A (2016) Bioenergetic roles of mitochondrial fusion. Biochim Biophys Acta 1857:1277-83.MouseFibroblast
Kawalec 2016 Praca doktorska2016Kawalec MM (2016) Badanie komórkowych skutków mutacji w genie mitofuzyny 2. Próba zaproponowania testu patogennoƛci mutacji. Praca doktorska p86.MouseFibroblast
Heimer 2016 Am J Hum Genet2016Heimer G, KerÀtÀr JM, Riley LG, Balasubramaniam S, Eyal E, PietikÀinen LP, Hiltunen JK, Marek-Yagel D, Hamada J, Gregory A, Rogers C, Hogarth P, Nance MA, Shalva N, Veber A, Tzadok M, Nissenkorn A, Tonduti D, Renaldo F, Kraoua I, Panteghini C, Valletta L, Garavaglia B, Cowley MJ, Gayevskiy V, Roscioli T, Silberstein JM, Hoffmann C, Raas-Rothschild A, Tiranti V, Anikster Y, Christodoulou J, Kastaniotis AJ, Ben-Zeev B, Hayflick SJ (2016) MECR mutations cause childhood-onset dystonia and optic atrophy, a mitochondrial fatty acid synthesis disorder. Am J Hum Genet 99:1229-44.HumanFibroblast
Schoepf 2016 FEBS J2016Schöpf B, SchÀfer G, Weber A, Talasz H, Eder IE, Klocker H, Gnaiger E (2016) Oxidative phosphorylation and mitochondrial function differ between human prostate tissue and cultured cells. https://doi.org/10.1111/febs.13733HumanEndothelial;epithelial;mesothelial cell
Genital
Other cell lines
Fibroblast
Alvarez-Mora 2016 Mol Neurobiol2016Alvarez-Mora MI, Rodriguez-Revenga L, Madrigal I, Guitart-Mampel M, Garrabou G, MilĂ  M (2016) Impaired mitochondrial function and dynamics in the pathogenesis of FXTAS. Mol Neurobiol 54:6896-902.HumanFibroblast
Macheiner 2016 Sci Rep2016Macheiner T, Fengler VH, Agreiter M, Eisenberg T, Madeo F, Kolb D, Huppertz B, Ackbar R, Sargsyan K (2016) Magnetomitotransfer: An efficient way for direct mitochondria transfer into cultured human cells. Sci Rep 6:35571.HumanFibroblast
Lung;gill
Ehinger 2016 Nat Commun2016Ehinger JK, Piel S, Ford R, Karlsson M, Sjövall F, Frostner EÅ, Morota S, Taylor RW, Turnbull DM, Cornell C, Moss SJ, Metzsch C, Hansson MJ, Fliri H, ElmĂ©r E (2016) Cell-permeable succinate prodrugs bypass mitochondrial Complex I deficiency. Nat Commun 7:12317. https://doi.org/10.1038/ncomms12317Heart
Blood cells
Fibroblast
Scrima 2016 Biochim Biophys Acta2016Scrima R, Cela O, Merla G, Augello B, Rubino R, Quarato G, Fugetto S, Menga M, Fuhr L, RelĂłgio A, Piccoli C, Mazzoccoli G, Capitanio N (2016) Clock-genes and mitochondrial respiratory activity: Evidence of a reciprocal interplay. Biochim Biophys Acta 1857:1344-51.HumanLiver
Endothelial;epithelial;mesothelial cell
Other cell lines
Fibroblast
Sengupta 2016 Free Radic Biol Med2016Sengupta S, Yang G, O'Donnell JC, Hinson MD, McCormack SE, Falk MJ, La P, Robinson MB, Williams ML, Yohannes MT, Polyak E, Nakamaru-Ogiso E, Dennery PA (2016) The circadian gene Rev-erbα improves cellular bioenergetics and provides preconditioning for protection against oxidative stress. Free Radic Biol Med 93:177-89.MouseLung;gill
Fibroblast
Sheremet 2016 Biochemistry (Moscow)2016Sheremet NL, Nevinitsyna TA, Zhorzholadze NV, Ronzina IA, Itkis YS, Krylova TD, Tsygankova PG, Malakhova VA, Zakharova EY, Tokarchuk AV, Panteleeva AA, Karger EM, Lyamzaev KG, Avetisov SE (2016) Previously unclassified mutation of mtDNA m.3472T>C: evidence of pathogenicity in Leber’s hereditary optic neuropathy. Biochemistry (Moscow) BM16-078.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Platelet
Torres 2016 Redox Biol2016Torres S, Matías N, Baulies A, Nuñez S, Alarcon-Vila C, Martinez L, Nuño N, Fernandez A, Caballeria J, Levade T, Gonzalez-Franquesa A, Garcia-Rovés P, Balboa E, Zanlungo S, Fabrías G, Casas J, Enrich C, Garcia-Ruiz C, Fernåndez-Checa JC (2016) Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease. Redox Biol 11:60-72.Human
Mouse
Nervous system
Liver
Endothelial;epithelial;mesothelial cell
Fibroblast
Peruzzo 2016 Cell Cycle2016Peruzzo P, Comelli M, Di Giorgio E, Franforte E, Mavelli I, Brancolini C (2016) Transformation by different oncogenes relies on specific metabolic adaptations. Cell Cycle 15:2656-68.MouseFibroblast
Franko 2016 Diabetes2016Franko A, Huypens P, Neschen S, Irmler M, Rozman J, Rathkolb B, Neff F, Prehn C, Dubois G, Baumann M, Massinger R, Gradinger D, Przemeck GK, Repp B, Aichler M, Feuchtinger A, Schommers P, Stöhr O, Sanchez-Lasheras C, Adamski J, Peter A, Prokisch H, Beckers J, Walch AK, Fuchs H, Wolf E, Schubert M, Wiesner RJ, Hrabě de Angelis M (2016) Bezafibrate improves insulin sensitivity and metabolic flexibility in STZ-treated diabetic mice. Diabetes 65:2540-52.HumanSkeletal muscle
Fibroblast
Gerber 2016 J Med Genet2016Gerber S, Ding MG, GĂ©rard X, Zwicker K, Zanlonghi X, Rio M, Serre V, Hanein S, Munnich A, Rotig A, Bianchi L, Amati-Bonneau P, Elpeleg O, Kaplan J, Brandt U, Rozet JM (2016) Compound heterozygosity for severe and hypomorphic NDUFS2 mutations cause non-syndromic LHON-like optic neuropathy. J Med Genet 54:346-56.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Leman 2015 Int J Biochem Cell Biol2015Leman G, Gueguen N, Desquiret-Dumas V, Kane MS, Wettervald C, Chupin S, Chevrollier A, Lebre AS, Bonnefont JP, Barth M, Amati-Bonneau P, Verny C, Henrion D, Bonneau D, Reynier P, Procaccio V (2015) Assembly defects induce oxidative stress in inherited mitochondrial complex I deficiency. Int J Biochem Cell Biol 65:91-103.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Mourier 2015 J Cell Biol2015Mourier A, Motori E, Brandt T, Lagouge M, Atanassov I, Galinier A, Rappl G, Brodesser S, Hultenby K, Dieterich C, Larsson NG (2015) Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels. J Cell Biol 208:429-42.MouseHeart
Fibroblast
Plecita-Hlavata 2015 Am J Respir Cell Mol Biol2015PlecitĂĄ-HlavatĂĄ L, Tauber J, Li M, Zhang H, Flockton AR, Pullamsetti SS, Chelladurai P, D'alessandro A, El Kasmi KC, JeĆŸek P, Stenmark KR (2015) Constitutive reprogramming of fibroblast mitochondrial metabolism in pulmonary hypertension. Am J Respir Cell Mol Biol 55:47-57.Human
Bovines
Endothelial;epithelial;mesothelial cell
Fibroblast
Kawalec 2015 PLoS One2015Kawalec M, BoratyƄska-JasiƄska A, Beręsewicz M, Dymkowska D, ZabƂocki K, ZabƂocka B (2015) Mitofusin 2 deficiency affects energy metabolism and mitochondrial biogenesis in MEF cells. PLoS One 10:e0134162.MouseFibroblast
Lim 2015 Am J Cancer Res2015Lim SC, Carey KT, McKenzie M (2015) Anti-cancer analogues ME-143 and ME-344 exert toxicity by directly inhibiting mitochondrial NADH: ubiquinone oxidoreductase (Complex I). Am J Cancer Res 5:689-701.HumanKidney
Other cell lines
HEK
HeLa
Fibroblast
Garama 2015 Mol Cell Biol2015Garama DJ, Harris TJ, White CL, Rossello FJ, Abdul-Hay M, Gough DJ, Levy DE (2015) A synthetic lethal interaction between glutathione synthesis and mitochondrial reactive oxygen species provides a tumor-specific vulnerability dependent on STAT3. Mol Cell Biol 35:3646-56.MouseEndothelial;epithelial;mesothelial cell
Fibroblast
Seillier 2015 EMBO Mol Med2015Seillier M, Pouyet L, N'Guessan P, Nollet M, Capo F, Guillaumond F, Peyta L, Dumas JF, Varrault A, Bertrand G, Bonnafous S, Tran A, Meur G, Marchetti P, Ravier MA, Dalle S, Gual P, Muller D, Rutter GA, Servais S, Iovanna JL, Carrier A (2015) Defects in mitophagy promote redox-driven metabolic syndrome in the absence of TP53INP1. EMBO Mol Med 7:802-18.Human
Mouse
Fibroblast
Kishita 2015 Am J Hum Genet2015Kishita Y, Pajak A, Bolar NA, Marobbio CM, Maffezzini C, Miniero DV, Monné M, Kohda M, Stranneheim H, Murayama K, Naess K, Lesko N, Bruhn H, Mourier A, Wibom R, Nennesmo I, Jespers A, Govaert P, Ohtake A, Van Laer L, Loeys BL, Freyer C, Palmieri F, Wredenberg A, Okazaki Y, Wedell A (2015) Intra-mitochondrial methylation deficiency due to mutations in SLC25A26. Am J Hum Genet 97:761-8.HumanSkeletal muscle
Fibroblast
Kluckova 2015 Cell Death Dis2015Kluckova K, Sticha M, Cerny J, Mracek T, Dong L, Drahota Z, Gottlieb E, Neuzil J, Rohlena J (2015) Ubiquinone-binding site mutagenesis reveals the role of mitochondrial complex II in cell death initiation. Cell Death Dis 6:e1749.Other mammalsLung;gill
Other cell lines
Fibroblast
Angebault 2015 Am J Hum Genet2015Angebault C, Guichet PO, Talmat-Amar Y, Charif M, Gerber S, Fares-Taie L, Gueguen N, Halloy F, Moore D, Amati-Bonneau P, Manes G, Hebrard M, Bocquet B, Quiles M, Piro-MĂ©gy C, Teigell M, Delettre C, Rossel M, Meunier I, Preising M, Lorenz B, Carelli V, Chinnery PF, Yu-Wai-Man P, Kaplan J, Roubertie A, Barakat A, Bonneau D, Reynier P, Rozet JM, Bomont P, Hamel CP, Lenaers G (2015) Recessive mutations in RTN4IP1 cause isolated and syndromic optic neuropathies. Am J Hum Genet 97:754-60.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Zelenka 2015 Oxid Med Cell Longev2015Zelenka J, Dvoƙák A, Alán L (2015) L-lactate protects skin fibroblasts against aging-associated mitochondrial dysfunction via mitohormesis. Oxid Med Cell Longev 2015:351698.RatLiver
Endothelial;epithelial;mesothelial cell
Fibroblast
Abou-Hamdan 2015 Methods Enzymol2015Abou-Hamdan A, Guedouari-Bounihi H, Lenoir V, Andriamihaja M, Blachier F, Bouillaud F (2015) Oxidation of H2S in mammalian cells and mitochondria. Methods Enzymol 554:201-28.Human
Pig
Mouse
Rat
Heart
Nervous system
Liver
Kidney
Genital
CHO
Neuroblastoma
Fibroblast
Logan 2014 Nat Genet2014Logan CV, Szabadkai G, Sharpe JA, Parry DA, Torelli S, Childs AM, Kriek M, Phadke R, Johnson CA, Roberts NY, Bonthron DT, Pysden KA, Whyte T, Munteanu I, Foley AR, Wheway G, Szymanska K, Natarajan S, Abdelhamed ZA, Morgan JE, Roper H, Santen GW, Niks EH, van der Pol WL, Lindhout D, Raffaello A, De Stefani D, den Dunnen JT, Sun Y, Ginjaar I, Sewry CA, Hurles M, Rizzuto R. UK10K Consortium, Duchen MR, Muntoni F, Sheridan E (2014) Loss-of-function mutations in MICU1 cause a brain and muscle disorder linked to primary alterations in mitochondrial calcium signaling. Nat Genet 46:188-93.HumanFibroblast
Jackson 2014 J Med Genet2014Jackson CB, Nuoffer JM, Hahn D, Prokisch H, Haberberger B, Gautsch M, HĂ€berli A, Gallati S, Schaller A (2014) Mutations in SDHD lead to autosomal recessive encephalomyopathy and isolated mitochondrial Complex II deficiency. J Med Genet 51:170-5.HumanFibroblast
Gnaiger 2014 MitoPathways2014
O2k-Protocols
Gnaiger E (2014) Mitochondrial pathways and respiratory control. An introduction to OXPHOS analysis. 4th ed. Mitochondr Physiol Network 19.12. Oroboros MiPNet Publications, Innsbruck:80 pp. — see 5th edition: Gnaiger 2020 BEC MitoPathways.
Human
Mouse
Heart
Skeletal muscle
Fibroblast
Ye 2013 Anal Biochem2013Ye F, Hoppel CL (2013) Measuring oxidative phosphorylation in human skin fibroblasts. https://doi.org/10.1016/j.ab.2013.02.010HumanSkeletal muscle
Fibroblast
Zhang 2013 PLoS One2013Zhang Z, Tsukikawa M, Peng M, Polyak E, Nakamaru-Ogiso E, Ostrovsky J, McCormack S, Place E, Clarke C, Reiner G, McCormick E, Rappaport E, Haas R, Baur JA, Falk MJ (2013) Primary respiratory chain disease causes tissue-specific dysregulation of the global transcriptome and nutrient-sensing signaling network. PLoS One 8:e69282.HumanFibroblast
Huai 2013 J Cell Sci2013Huai J, Vögtle FN, Jöckel L, Li Y, Kiefer T, Ricci JE, Borner C (2013) TNFα-induced lysosomal membrane permeability (LMP) is downstream of MOMP and triggered by caspase-mediated p75 cleavage and ROS formation. J Cell Sci 126:4015-25.Human
Mouse
HeLa
Fibroblast
Goo 2012 PLoS One2012Goo CK, Lim HY, Ho QS, Too HP, Clement MV, Wong KP (2012) PTEN/Akt signaling controls mitochondrial respiratory capacity through 4E-BP1. PLoS One 7:e45806.MouseFibroblast
Iyer 2012 Hum Gene Ther2012Iyer S, Bergquist K, Young K, Gnaiger E, Rao RR, Bennett JP Jr (2012) Mitochondrial gene therapy improves respiration, biogenesis and transcription in G11778A Leber’s hereditary optic neuropathy and T8993G Leigh’s syndrome cells. Hum Gene Ther 23:647-57.HumanFibroblast
El-Bacha 2012 Int J Biochem Cell Biol2012El-Bacha T, Da Poian AT (2012) Virus-induced changes in mitochondrial bioenergetics as potential targets for therapy. Int J Biochem Cell Biol 45:41-6.Human
Mouse
Nervous system
Liver
Fibroblast
Pesta 2012 Methods Mol Biol2012Pesta D, Gnaiger E (2012) High-resolution respirometry. OXPHOS protocols for human cells and permeabilized fibers from small biopsies of human muscle. Methods Mol Biol 810:25-58. https://doi.org/10.1007/978-1-61779-382-0_3HumanSkeletal muscle
Other cell lines
HEK
Fibroblast
HUVEC
KozieƂ 2011 J Invest Dermatol2011KozieƂ R, Greussing R, Maier AB, Declercq L, Jansen-DĂŒrr P (2011) Functional interplay between mitochondrial and proteasome activity in skin aging. J Invest Dermatol 131:594-603.HumanFibroblast
Quarato 2011 Biochim Biophys Acta2011Quarato G, Piccoli C, Scrima R, Capitanio N (2011) Variation of flux control coefficient of cytochrome c oxidase and of the other respiratory chain complexes at different values of protonmotive force occurs by a threshold mechanism. Biochim Biophys Acta 1807: 1114-1124.Human
Mouse
Liver
Fibroblast
Paulsen 2011 Cell Transplant2011Paulsen BD, Maciel RD, Galina A, da Silveira MS, Souza CD, Drummond H, Pozzato EN, Junior HS, Chicaybam L, Massuda R, Setti-PerdigĂŁo P, Bonamino M, Belmonte-de-Abreu PS, Castro NG, Brentani H, Rehen SK (2011) Altered oxygen metabolism associated to neurogenesis of induced pluripotent stem cells derived from a schizophrenic patient. Cell Transplant 21:1547-59HumanFibroblast
Tello 2011 Cell Metab2011Tello D, Balsa E, Acosta-Iborra B, Fuertes-Yebra E, Elorza A, Ordóñez Á, Corral-Escariz M, Soro I, López-Bernardo E, Perales-Clemente E, Martínez-Ruiz A, Enríquez JA, Aragonés J, Cadenas S, Landåzuri MO (2011) Induction of the mitochondrial NDUFA4L2 protein by HIF-1α decreases oxygen consumption by inhibiting Complex I activity. Cell Metab 14:768-79.MouseFibroblast
Ye 2011 J Biol Chem2011Ye F, Lemieux H, Hoppel CL, Hanson RW, Hakimi P, Croniger CM, Puchowicz M, Anderson VE, Fujioka H, Stavnezer E (2011) Peroxisome proliferator-activated receptor Îł (PPARÎł) mediates a Ski-induced shift from glycolysis to oxidative energy metabolism. J Biol Chem 286:40013-24.ChickenFibroblast
Thomas 2011 Mitochondrion2011Thomas RR, Khan SM, Portell FR, Smigrodzki RM, Bennett JP Jr (2011) Recombinant human mitochondrial transcription factor A stimulates mitochondrial biogenesis and ATP synthesis, improves motor function after MPTP, reduces oxidative stress and increases survival after endotoxin. Mitochondrion 11:108-18.Human
Mouse
Heart
Skeletal muscle
Nervous system
Fibroblast
Remor 2011 Biochim Biophys Acta2011Remor AP, de Matos FJ, Ghisoni K, da Silva TL, Eidt G, BĂșrigo M, de Bem AF, Silveira PC, de LeĂłn A, Sanchez MC, Hohl A, Glaser V, Saraiva-Gonçalves CA, Quincozes-Santos A, Borba Rosa R, Latini A (2011) Differential effects of insulin on peripheral diabetes-related changes in mitochondrial bioenergetics: Involvement of advanced glycosylated end products. Biochim Biophys Acta 1812:1460-71.RatEndothelial;epithelial;mesothelial cell
Fibroblast
Smolkova 2010 J Bioenerg Biomembr2010SmolkovĂĄ K, Bellance N, Scandurra F, GĂ©not E, Gnaiger E, PlecitĂĄ-HlavatĂĄ L, JeĆŸek P, Rossignol R (2010) Mitochondrial bioenergetic adaptations of breast cancer cells to aglycemia and hypoxia. https://doi.org/10.1007/s10863-009-9267-xHumanFibroblast
Guillet 2010 Mitochondrion2010Guillet V, Chevrollier A, Cassereau J, Letournel F, Gueguen N, Richard L, Desquiret V, Verny C, Procaccio V, Amati-Bonneau P, Reynier P, Bonneau D (2010) Ethambutol-induced optic neuropathy linked to OPA1 mutation and mitochondrial toxicity. Mitochondrion 10:115-24.HumanFibroblast
Krebiehl 2010 PLoS One2010Krebiehl G, Ruckerbauer S, Burbulla LF, Kieper N, Maurer B, Waak J, Wolburg H, Gizatullina Z, Gellerich FN, Woitalla D, Riess O, Kahle PJ, Proikas-Cezanne T, Krueger R (2010) Reduced basal autophagy and impaired mitochondrial dynamics due to loss of Parkinson's disease-associated protein DJ-1. PLoS One 5:e9367.MouseFibroblast
Casasnovas 2009 J Med Genet2010Casasnovas C, Banchs I, Cassereau J, Gueguen N, Chevrollier A, MartĂ­nez-Matos JA, Bonneau D, Volpini V (2010) Phenotypic spectrum of MFN2 mutations in the Spanish population. J Med Genet 47:249-56.HumanNervous system
Fibroblast
Fornuskova 2010 Thesis2010FornĆŻskovĂĄ D (2010) Biochemical and molecular studies of cytochrome c oxidase and ATP synthase deficiencies. PhD Thesis p93.HumanHeart
Skeletal muscle
Nervous system
Liver
HEK
Fibroblast
Kotarsky 2010 Mitochondrion2010Kotarsky H, Karikoski R, Mörgelin M, Marjavaara S, Bergman P, Zhang DL, Smet J, van Coster R, Fellman V (2010) Characterization of complex III deficiency and liver dysfunction in GRACILE syndrome caused by a BCS1L mutation. Mitochondrion 10:497-509.HumanFibroblast
Guillet 2010 neurogenetics2010Guillet V, Gueguen N, Verny C, Ferre M, Homedan C, Loiseau D, Procaccio V, Amati-Bonneau P, Bonneau D, Reynier P, Chevrollier A (2010) Adenine nucleotide translocase is involved in a mitochondrial coupling defect in MFN2-related Charcot-Marie-Tooth type 2A disease. Neurogenetics 11:127-33.HumanFibroblast
Baron 2010 Thesis2010Baron M (2010) Copy number variations of the mitochondrial DNA as potential cause of mitochondrial diseases. Thesis Rheinischen Friedrich-Wilhelms-Universitaet Bonn, Mathematisch-Naturwissenschaftlichen FakultÀt, 110pp.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Yang 2010 Cancer Biol Ther2010Yang D, Wang MT, Tang Yong, Chen Y, Jiang H, Jones TT, Rao K, Brewer GJ, Singh KK, Nie D (2010) Impairment of mitochondrial respiration in mouse fibroblasts by oncogenic H-RAS(Q61L). Canc Biol Ther 9:122-33.MouseFibroblast
Haack 2010 Nat Genet2010Haack TB, Danhauser K, Haberberger B, Hoser J, Strecker V, Boehm D, Uziel G, Lamantea E, Invernizzi F, Poulton J, Rolinski B, Iuso A, Biskup S, Schmidt T, Mewes HW, Wittig I, Meitinger T, Zeviani M, Prokisch H (2010) Exome sequencing identifies ACAD9 mutations as a cause of Complex I deficiency. Nat Genet 42:1131-4.Fibroblast
Dikov 2010 Exp Gerontol2010Dikov D, Aulbach A, Muster B, Dröse S, Jendrach M, Bereiter-Hahn J (2010) Do UCP2 and mild uncoupling improve longevity? Exp Gerontol 45:586-95.HeLa
Fibroblast
HUVEC
Bellance 2009 Int J Biochem Cell Biol2009Bellance N, Benard G, Furt F, Begueret H, SmolkovĂĄ K, Passerieux E, Delage JP, Baste JM, Moreau P, Rossignol R (2009) Bioenergetics of lung tumors: Alteration of mitochondrial biogenesis and respiratory capacity. Int J Biochem Cell Biol 41:2566-77.HumanFibroblast
Mufazalov 2009 Mol Biol (Mosk)2009Mufazalov IA, Pen'kov DN, Cherniak BV, Pletiushkina OIu, Vysokikh MIu, Kirpichnikov MP, Dolgikh DA, Kruglov AA, Kuprash DV, Skulachev VP, Nedospasov SA (2009) Preparation and characterization of mouse embryonic fibroblasts with K72W mutation in somatic cytochrome c gene. Mol Biol (Mosk) 43:648-56.MouseFibroblast
Nochez 2009 Mol Vis2009Nochez Y, Arsene S, Gueguen N, Chevrollier A, Ferré M, Guillet V, Desquiret V, Toutain A, Bonneau D, Procaccio V, Amati-Bonneau P, Pisella PJ, Reynier P (2009) Acute and late-onset optic atrophy due to a novel OPA1 mutation leading to a mitochondrial coupling defect. Mol Vis 15:598-608.HumanFibroblast
Haendeler 2009 Arterioscler Thromb Vasc Biol2009Haendeler J, Dröse S, BĂŒchner N, Jakob S, Altschmied J, Goy C, Spyridopoulos I, Zeiher AM, Brandt U, Dimmeler S (2009) Mitochondrial telomerase reverse transcriptase binds to and protects mitochondrial DNA and function from damage. Arterioscler Thromb Vasc Biol 29:929-35.Human
Mouse
Heart
Endothelial;epithelial;mesothelial cell
Fibroblast
Hoepken 2007 Neurobiol Dis2007Hoepken HH, Gispert S, Morales B, Wingerter O, Del Turco D, MĂŒlsch A, Nussbaum RL, MĂŒller K, Dröse S, Brandt U, Deller T, Wirth B, Kudin AP, Kunz WS, Auburger G (2007) Mitochondrial dysfunction, peroxidation damage and changes in glutathione metabolism in PARK6. Neurobiol Dis 25:401-11.HumanFibroblast
Rodriguez-Juarez 2007 Biochem J2007Rodriguez-Juarez F, Aguirre E, Cadenas S (2007) Relative sensitivity of soluble guanylate cyclase and mitochondrial respiration to endogenous nitric oxide at physiological oxygen concentration. Biochem J 405:223-31.RatFibroblast
Blakely 2006 Pediatr Res2006Blakely EL, Rennie KJ, Jones L, Elstner M, Chrzanowska-Lightowlers ZMA, White CB, Shield JPH, Pilz DT, Turnbull DM, Poulton J, Taylor RW (2006) Sporadic intragenic inversion of the mitochondrial DNA MTND1 gene causing fatal infantile lactic acidosis. Pediatr Res 59:440-4.HumanFibroblast
Deschauer 2006 Mol Genet Metab2006Deschauer M, Gizatullina Z, Schulze A, Pritsch M, Knöppel C, Knape M, Zierz S, Gellerich FN (2006) Molecular and biochemical investigations in fumarase deficiency. Mol Genet Metab 88:146-52.HumanFibroblast
Garedew 2005 Eur Shock Soc2005Garedew A, HĂŒtter E, Haffner B, Gradl P, Gradl L, Jansen-DĂŒrr P, Gnaiger E (2005) High-resolution respirometry for the study of mitochondrial function in health and disease. The Oroboros O2k. Proc 11th Congress Eur Shock Soc, Vienna, Austria (Redl H, ed) Medimond, Bologna:107-11.HumanFibroblast
Oezen 2005 Hum Mol Genet2005Oezen I, Rossmanith W, Forss-Petter S, Kemp S, Voigtlander T, Moser-Thier K, Wanders RJA, Bittner RE, Berger J (2005) Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency. Hum Mol Genet 14:1127-37.Human
Mouse
Skeletal muscle
Nervous system
Fibroblast
Winkler-Stuck 2004 J Neurol Sci2004Winkler-Stuck K, Wiedemann FR, Wallesch CW, Kunz WS (2004) Effect of coenzyme Q10 on the mitochondrial function of skin fibroblasts from Parkinson patients. J Neurol Sci 220:41-8.HumanFibroblast
Huetter 2004 Biochem J2004HĂŒtter E, Renner K, Pfister G, Stöckl P, Jansen-DĂŒrr P, Gnaiger E (2004) Senescence-associated changes in respiration and oxidative phosphorylation in primary human fibroblasts. https://doi.org/10.1042/BJ20040095HumanFibroblast
Jesina 2004 Biochem J2004Jesina P, Tesarova M, Fornuskova D, Vojtiskova A, Pecina P, Kaplanova V, Hansikova H, Zeman J, Houstek J (2004) Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206. Biochem J 383:561-71.HumanFibroblast
Pecina 2004 Am J Physiol Cell Physiol2004Pecina P, Gnaiger E, Zeman J, Pronicka E, Houstek J (2004) Decreased affinity to oxygen of cytochrome c oxidase in Leigh syndrome caused by SURF1 mutations. Am J Physiol Cell Physiol 287:C1384-8.HumanFibroblast
Mayr 2004 Pediatr Res2004Mayr JA, Paul J, Pecina P, Kurnik P, Forster H, Fotschl U, Sperl W, Houstek J (2004) Reduced respiratory control with ADP and changed pattern of respiratory chain enzymes as a result of selective deficiency of the mitochondrial ATP synthase. Pediatr Res 55:988-94.HumanHeart
Skeletal muscle
Fibroblast
Pecina 2004 Physiol Res2004Pecina P, Houstkova H, Hansikova H, Zeman J, Houstek J (2004) Genetic defects of cytochrome c oxidase assembly. Physiol Res 53 Suppl 1:S213-23.HumanSkeletal muscle
Fibroblast
Pecina 2003 Biochim Biophys Acta2003Pecina P, Capkova M, Chowdhury SK, Drahota Z, Dubot A, Vojtiskova A, Hansikova H, Houstekova H, Zeman J, Godinot C, Houstek J (2003) Functional alteration of cytochrome c oxidase by SURF1 mutations in Leigh syndrome. Biochim Biophys Acta 1639:53-63.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Gnaiger 2003 Adv Exp Med Biol2003Gnaiger E (2003) Oxygen conformance of cellular respiration. A perspective of mitochondrial physiology. https://doi.org/10.1007/978-1-4419-8997-0_4Human
Rat
Heart
Liver
Endothelial;epithelial;mesothelial cell
Fibroblast
Huetter 2002 Mol Biol Rep2002HĂŒtter E, Renner K, Jansen-DĂŒrr P, Gnaiger E (2002) Biphasic oxygen kinetics of cellular respiration and linear oxygen dependence of antimycin A inhibited oxygen consumption. Mol Biol Rep 29:83-7.HumanFibroblast
Chowdhury 2000 Clin Chim Acta2000Chowdhury SK, Drahota Z, Floryk D, Calda P, Houstek J (2000) Activities of mitochondrial oxidative phosphorylation enzymes in cultured amniocytes. Clin Chim Acta 298:157-73.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Messer 2000 J Biomed Materials Res2000Messer RLW, Doeller JE, Kraus DW, Lucas LC (2000) An investigation of fibroblast mitochondria enzyme activity and respiration in response to metallic ions released from dental alloys. J Biomed Materials Res 50:598-604.HumanFibroblast
O2k-Publications in the MiPMap

O2k-Publications: Fibroblast - Abstracts

Sort in ascending/descending order by a click on one of the small symbols in squares below.
 ReferenceOrganismTissue;cell
Gnaiger 2022 ESCI Bari
Erich Gnaiger
Gnaiger E (2022) Bioenergetic cluster analysis – diagnostic evaluation of mitochondrial respiratory control in human fibroblasts. 56th Annual Scientific Meeting of the European Society for Clinical Investigation.
HumanFibroblast
Gnaiger 2019 NatureConferenceGnaiger E, Passrugger M, Gallee L, Felipe F Garcia-Souza, Zdrazilova L, Doerrier C (2019) High-resolution respirometry and innovations in the Oroboros O2k-technology.HumanBlood cells
HEK
Fibroblast
Platelet
Zdrazilova 2018 MiPschool Tromso C4
MiPsociety
Bioenergetic characterization of skin fibroblasts from patients with congenital disorders of glycosylation.
HumanFibroblast
Luis-Garcia 2018 Am J Respir Crit Care MedContribution of the mitochondrial intrinsic pathway to the apoptosis resistance exhibited by fibroblasts from idiopathic pulmonary fibrosis.HumanLung;gill
Fibroblast
Gnaiger 2017 Abstract EUROMIT2017 Cologne
EUROMIT2017
Again not a Warburg effect - mitochondrial function in primary prostate cancer.
HumanEndothelial;epithelial;mesothelial cell
Genital
Fibroblast
Krylova 2017 MiPschool Obergurgl
KrylovaT.jpg
High-resolution respirometry in diagnostic of mitochondrial complex I deficiency.
Endothelial;epithelial;mesothelial cell
Fibroblast
Iyer 2017 MiP2017
Iyer Shilpa
Bioenergetic profiling of patient derived human fibroblasts for mitochondrial diseases. Iyer_Presentation
HumanFibroblast
Aasander Frostner 2017 MiP2017
Åsander Frostner Eleonor
Cell-permeable succinate and malonate as research tools. Aasander Frostner_Poster
HumanHeart
Liver
Blood cells
Fibroblast
Gnaiger 2016 Abstract UMDF2016Schöpf B, SchÀfer G, Weber A, Talasz H, Eder IE, Klocker H, Gnaiger E (2016) Oxidative phosphorylation and mitochondrial function differ between human prostate tissue and cultured cells.HumanEndothelial;epithelial;mesothelial cell
Genital
Other cell lines
Fibroblast
Hoppel 2016c Abstract MitoFit Science Camp 2016Mitochondrial respiration in permeabilized skin fibroblasts.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Bird 2016 Abstract IOC115High-resolution respirometry for the diagnosis of mitochondrial disease.HumanSkeletal muscle
Liver
Fibroblast
Ost 2015 Abstract IOC100Ost M (2015) “Mitokines” and the organismal role of mitochondrial function in energy homeostasis & metabolism. Mitochondr Physiol Network 20.01.MouseSkeletal muscle
Fibroblast
Ehinger 2015 Abstract MiPschool London 2015Cell-permeable mitochondrial complex II substrates – a new compound class for treatment of complex I-linked mitochondrial disease.HumanHeart
Blood cells
Fibroblast
Logan 2015 Abstract MiPschool London 2015Loss-of-function mutations in MICU1 cause a brain and muscle disorder linked to primary alterations in mitochondrial calcium signalling.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Hahn 2014 Abstract MiP2014Validation of oxygen consumption measurements in muscle and fibroblasts from patients with mitochondrial diseases.HumanSkeletal muscle
Fibroblast
Wagenaars 2014 Abstract IOC 2014-04 SchroeckenWagenaars J, Willems PHGM, Koopman WJ (2014) Oxygen consumption in OXPHOS-deficient cells. Mitochondr Physiol Network 19.02.Human
Mouse
Skeletal muscle
Fibroblast
Bristot 2014 Abstract MiP2014High-resolution respirometry and bioenergetics evaluation in fibroblasts derived from patients with TP53 germline mutations.HumanFibroblast
Dohlmann 2014 Abstract MiP2014Mitochondrial adaptations to high-intensity training in young and elderly men and women.HumanSkeletal muscle
HeLa
Fibroblast
Felser 2014 Abstract MiP2014Assessing mitochondrial dysfunction in fibroblast cells – a comparison between O2k and multiwell respirometry.HumanEndothelial;epithelial;mesothelial cell
HeLa
Fibroblast
Wieckowski 2013 Abstract MiP2013Wieckowski MR, Karkucinska-Wieckowska A, Wojtala A, Lebiedzinska M, Pronicki M, Duszynski J (2013) Mitochondrial bioenergetic parameters, reactive oxygen species production and the status of antioxidant defense system can be used to differentiate mitochondrial defects studied in the fibroblasts from patients with various mitochondrial disorders. Mitochondr Physiol Network 18.08.Human
Mouse
Fibroblast
Kallijarvi 2013 Abstract IOC75Kallijarvi J (2013) Respiratory chain dysfunction in the Complex III assembly disorder GRACILE. Mitochondr Physiol Network 18.03.Human
Mouse
Liver
Kidney
Fibroblast
Skulachev 2013 Abstract MiP2013Skulachev VP (2013) SkQ1, the first mitochondria-targeted medicine available in drugstores. Mitochondr Physiol Network 18.08.Human
Mouse
Horse
Dog
Rabbit
Fishes
Other invertebrates
Fungi
Plants
Heart
Skeletal muscle
Nervous system
Kidney
Islet cell;pancreas;thymus
Other cell lines
HeLa
Fibroblast
Stem cells
Iyer 2012 Abstract BioblastIyer S (2012) Gentle Science in the real world of mitochondrial physiology and genetics. Mitochondr Physiol Network 17.12.HumanFibroblast
Labieniec-Watala 2012 Abstract BioblastLabieniec-Watala M, Siewiera K, Watala C (2012) Seasonal fluctuations affect the physiology of laboratory animals – facts and artifacts from the perspective of heart mitochondrial respiratory response to tested compounds. Mitochondr Physiol Network 17.12.Mouse
Rat
Heart
Liver
Fibroblast
Nuskova 2011 Abstract IOC61Nuskova H, Pecina P, Kovarova N, Dell’Agnello C, Zeviani M, Houstek J (2011) Cytochrome c oxidase with decreased H+/e− ratio in SURF1 knockout mice. MiPNet16.01.MouseEndothelial;epithelial;mesothelial cell
Fibroblast
Marelsson 2011 Abstract IOC61Marelsson S (2011) Mitochondrial dysfunction in children with unknown encephalopathy. MiPNet16.01.HumanBlood cells
Fibroblast
Amoedo 2010 Abstract MiP2010AmoĂȘdo ND, Rodrigues MF, Pezutto P, Lourenço MVC, Madeiro da Costa RF, Oliveira MF, Galina A, Rumjanek FD, El-Bacha T (2010) Metabolic reprogramming of lung cancer cell induced by sodium butyrate: Studies on mitochondrial physiology and bioenergetics. Mitochondr Physiol Network 15.06.HumanEndothelial;epithelial;mesothelial cell
Fibroblast
Gnaiger Abstract MiP2010 2-01Gnaiger E (2010) Measuring the upper limit of mitochondrial performance - how and why?HumanFibroblast
Itkis 2010 Abstract IOC60Itkis YS (2010) Respirometry application in Leigh disease diagnostic and treatment. MiPNet15.10.HumanFibroblast

Comparative: tissues and cells

»Tissues, cell types and cell lines