Kunz WS

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Name Kunz Wolfram S, Prof., Dr.
Institution University Bonn, Department of Epileptology and

University Jena, Institute of Biochemistry II

Address Sigmund-Freud-Str. 25, 53127
City Bonn
State/Province
Country Germany
Email Wolfram.Kunz@ukb.uni-bonn.de
Weblink
O2k-Network Lab


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Publications

 PublishedReference
Augustynek 2018 Int J Mol Sci2018Augustynek B, Koprowski P, Rotko D, Kunz WS, Szewczyk A, Kulawiak B (2018) Mitochondrial BK channel openers CGS7181 and CGS7184 exhibit cytotoxic properties. Int J Mol Sci 19. pii: E353.
Kudin 2017 Int J Mol Sci2017Kudin AP, Mawasi H, Eisenkraft A, Elger CE, Bialer M, Kunz WS (2017) Mitochondrial liver toxicity of valproic acid and its acid derivatives is related to inhibition of α-lipoamide dehydrogenase. Int J Mol Sci 18. pii: E1912.
Vielhaber 2013 Acta Neuropathol2013Vielhaber S, Debska-Vielhaber G, Peeva V, Schoeler S, Kudin AP, Minin I, Schreiber S, Dengler R, Kollewe K, Zuschratter W, Kornblum C, Zsurka G, Kunz WS (2013) Mitofusin 2 mutations affect mitochondrial function by mitochondrial DNA depletion. Acta Neuropathol 125:245-56.
Franko 2012 J Mol Med2012Franko A, von Kleist-Retzow JC, Böse M, Sanchez-Lasheras C, Brodesser S, Krut O, Kunz WS, Wiedermann D, Höhn M, Stöhr O, Moll L, Freude S, Krone W, Schubert M, Wiesner RJ (2012) Complete failure of insulin-transmitted signaling, but not obesity-induced insulin resistance, impairs respiratory chain function in muscle. J Mol Med 90:1145-60.
Niehusmann 2011 Epilepsy Behav2011Niehusmann P, Surges R, von Wrede RD, Elger CE, Wellmer J, Reimann J, Urbach H, Vielhaber S, Bien CG, Kunz WS (2011) Mitochondrial dysfunction due to Leber's hereditary optic neuropathy as a cause of visual loss during assessment for epilepsy surgery. Epilepsy Behav 20:38-43.
Malinska 2010 Biochim Biophys Acta2010Malinska D, Kulawiak B, Kudin AP, Kovacs R, Huchzermeyer C, Kann O, Szewczyk A, Kunz WS (2010) Complex III-dependent superoxide production of brain mitochondria contributes to seizure-related ROS formation. Biochim Biophys Acta 1797:1163-70.
Skalska 2009 Int J Mol Sci2009Skalska J, Bednarczyk P, Piwonska M, Kulawiak B, Wilczynski G, Dolowy K, Kudin AP, Kunz WS, Szewczyk A (2009) Calcium ions regulate K uptake into brain mitochondria: The evidence for a novel potassium channel. Int J Mol Sci 10:1104-20.
Gispert 2009 PLoS One2009Gispert S, Ricciardi F, Kurz A, Azizov M, Hoepken HH, Becker D, Voos W, Leuner K, Müller WE, Kudin AP, Kunz WS, Zimmermann A, Roeper J, Wenzel D, Jendrach M, García-Arencíbia M, Fernández-Ruiz J, Huber L, Rohrer H, Barrera M, Reichert AS, Rüb U, Chen A, Nussbaum RL, Auburger G (2009) Parkinson phenotype in aged PINK1-deficient mice is accompanied by progressive mitochondrial dysfunction in absence of neurodegeneration. PLoS One 4:e5777.
Kuznetsov 2008 Nat Protoc2008Kuznetsov AV, Veksler V, Gellerich FN, Saks V, Margreiter R, Kunz WS (2008) Analysis of mitochondrial function in situ in permeabilized muscle fibers, tissues and cells. Nat Protoc 3:965-76.
Konieczny 2008 J Cell Biol2008Konieczny P, Fuchs P, Reipert S, Kunz WS, Zeöld A, Fischer I, Paulin D, Schröder R, Wiche G (2008) Myofiber integrity depends on desmin network targeting to Z-disks and costameres via distinct plectin isoforms. J Cell Biol 181:667-81.
Skalska 2008 Biochim Biophys Acta2008Skalska J, Piwonska M, Wyroba E, Surmacz L, Wieczorek R, Koszela-Piotrowska I, Zielinska J, Bednarczyk P, Dolowy K, Wilczynski GM, Szewczyk A, Kunz WS (2008) A novel potassium channel in skeletal muscle mitochondria. Biochim Biophys Acta 1777:651-9.
Kulawiak 2008 Exp Neurol2008Kulawiak B, Kudin AP, Szewczyk A, Kunz WS (2008) BK channel openers inhibit ROS production of isolated rat brain mitochondria. Exp Neurol 212:543-7.
Hoepken 2007 Neurobiol Dis2007Hoepken HH, Gispert S, Morales B, Wingerter O, Del Turco D, Mülsch A, Nussbaum RL, Müller 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.
Winkler-Stuck 2005 J Neural Transm2005Winkler-Stuck K, Kirches E, Mawrin C, Dietzmann K, Lins H, Wallesch CW, Kunz WS, Wiedemann FR (2005) Re-evaluation of the dysfunction of mitochondrial respiratory chain in skeletal muscle of patients with Parkinson's disease. J Neural Transm 112:499-518.
Kudin 2004 J Biol Chem2004Kudin AP, Bimpong-Buta NY, Vielhaber S, Elger CE, Kunz WS (2004) Characterization of superoxide-producing sites in isolated brain mitochondria. J Biol Chem 279:4127-35.
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.
Kuznetsov 2003 Anal Biochem2003Kuznetsov AV, Kunz WS, Saks V, Usson Y, Mazat J-P, Letellier T, Gellerich FN, Margreiter R (2003) Cryopreservation of mitochondria and mitochondrial function in cardiac and skeletal muscle fibers. Anal Biochem 319:296-303.
Vielhaber 2003 Epilepsia2003Vielhaber S, Von Oertzen JH, Kudin AF, Schoenfeld A, Menzel C, Biersack HJ, Kral T, Elger CE, Kunz WS (2003) Correlation of hippocampal glucose oxidation capacity and interictal FDG-PET in temporal lobe epilepsy. Epilepsia 44:193-9.
Debska 2002 Biochim Biophys Acta2002Debska G, Kicinska A, Skalska J, Szewczyk A, May R, Elger CE, Kunz WS (2002) Opening of potassium channels modulates mitochondrial function in rat skeletal muscle. Biochim Biophys Acta 1556:97-105.
Kudin 2002 Mol Biol Rep2002Kudin A, Vielhaber S, Elger CE, Kunz WS (2002) Differences in flux control and reserve capacity of cytochrome c oxidase (COX) in human skeletal muscle and brain suggest different metabolic effects of mild COX deficiencies. Mol Biol Rep 29:89-92.
Kudin 2002 Eur J Neurosci2002Kudin AP, Kudina TA, Seyfried J, Vielhaber S, Beck H, Elger CE, Kunz WS (2002) Seizure-dependent modulation of mitochondrial oxidative phosphorylation in rat hippocampus. Eur J Neurosci 15:1105-14.
Varlamov 2002 Hum Mol Genet2002Varlamov DA, Kudin AP, Vielhaber S, Schroder R, Sassen R, Becker A, Kunz D, Haug K, Rebstock J, Heils A, Elger CE, Kunz WS (2002) Metabolic consequences of a novel missense mutation of the mtDNA CO I gene. Hum Mol Genet 11:1797-805.
Wiedemann 2002 Recent Res Devel Anal Biochem2002Wiedemann FR, Winkler K, Kuznetsov AV, Halloul Z, Wallesch C-W, Kunz WS (2002) Recent Res Devel Anal Biochem 2:29-43.
Debska 2001 Brain Res2001Debska G, May R, Kicinska A, Swewczyk A, Elger C, Kunz WS (2001) Potassium channel openers depolarize hippocampal mitochondria. Brain Res 892:42-50.
Kunz 2000 J Biol Chem2000Kunz WS, Kudin A, Vielhaber S, Elger CE, Attardi G, Villani G (2000) Flux control of cytochrome c oxidase in human skeletal muscle. J Biol Chem 275:27741-5.
Kunz 2000 Ann Neurol2000Kunz WS, Kudin AP, Vielhaber S, Blumecke I, Zuschratter W, Schramm J, Beck H, Elger CE (2000) Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy. Ann Neurol 48:766-73.
Vielhaber 2000 Biochem Soc Trans2000Vielhaber S, Kudin A, Schroder R, Elger CE, Kunz WS (2000) Muscle fibres: applications for the study of the metabolic consequences of enzyme deficiencies in skeletal muscle. Biochem Soc Trans 28:159-64.
Kunz 1999 Brain Res1999Kunz WS, Goussakov IV, Beck H, Elger CE (1999) Altered mitochondrial oxidative phosphorylation in hippocampal slices of kainate-treated rats. Brain Res 826:236-42.
Kunz 1999 J Neurochem1999Kunz WS, Kuznetsov AV, Clark JF, Tracey I, Elger CE (1999) Metabolic consequences of the cytochrome c oxidase deficiency in brain of copper-deficient Movbr mice. J Neurochem 72:1580-5.
Vielhaber 1999 J Neurol Sci1999Vielhaber S, Winkler K, Kirches E, Kunz D, Büchner M, Feistner H, Elger CE, Ludolph AC, Riepe MW, Kunz WS (1999) Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis. J Neurol Sci 169:133-9.
Kudin 1999 Brain Res Brain Res Protoc1999Kudin A, Vielhaber S, Beck H, Elger CE, Kunz WS (1999) Quantitative investigation of mitochondrial function in single rat hippocampal slices: a novel application of high-resolution respirometry and laser-excited fluorescence spectroscopy. Brain Res Brain Res Protoc 4:329-34.
Kirches 1998 J Inherit Metab Dis1998Kirches EJF, Winkler K, Warich-Kirches M, Szibor R, Wien F, Kunz WS, von Bossanyi P, Bajaj PK, Dietzmann K (1998) mtDNA depletion and impairment of mitochondrial function in a case of a multisystem disorder including severe myopathy. J Inherit Metab Dis 21:400-8.
Saks 1998 Mol Cell Biochem1998Saks VA, Veksler VI, Kuznetsov AV, Kay L, Sikk P, Tiivel T, Tranqui L, Olivares J, Winkler K, Wiedemann F, Kunz WS (1998) Permeabilised cell and skinned fiber techniques in studies of mitochondrial function in vivo. Mol Cell Biochem 184:81-100.
Wiedemann 1998 FEBS Lett1998Wiedemann FR, Kunz WS (1998) Oxygen dependence of flux control of cytochrome c oxidase – implications for mitochondrial diseases. FEBS Lett 422:33-5.
Wiedemann 1998 J Neurol Sci1998Wiedemann FR, Winkler K, Kuznetsov AV, Bartels C, Vielhaber S, Feistner H, Kunz WS (1998) Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis. J Neurol Sci 156:65-72.
Kunz 1997 Anal Biochem1997Kunz D, Luley C, Winkler K, Lins H, Kunz WS (1997) Flow cytometric detection of mitochondrial dysfunction in subpopulations of human mononuclear cells. Anal Biochem 246:218-24.
Kunz 1997 Mol Cell Biochem1997Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW (1997) Detection of mitochondrial defects by laser fluorimetry. Mol Cell Biochem 174:97-100.
Kuznetsov 1997 Biochim Biophys Acta1997Kuznetsov AV, Winkler K, Kirches E, Lins H, Feistner H, Kunz WS (1997) Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases. Biochim Biophys Acta 1360:142-50.
Kuznetsov 1996 J Biol Chem1996Kuznetsov AV, Clark JF, Winkler K, Kunz WS (1996) Increase of flux control of cytochrome c oxidase in copper-deficient mottled brindled mice. J Biol Chem 271:283-8.
Kirches 1996 Saponin1996Kirches E, Winkler K, Kuznetsov AV, Lins H, von Bossanyi P, Dietzmann K, Frank B, Feistner H, Kunz WS (1996) Laserfluoreszenz-Studien der Mitochondrienfunktion in Saponin-permeabilisierten Skelettmuskelfasern von Patienten mit chronisch progressiver externer Ophthalmoplegie. Jahrb Neuromusk Erkrankungen 1996, Arcis Verlag:166-9.
Kuznetsov 1996 Jahrb Neuromusk Erkrankungen1996Kuznetsov AV, Clark JF, Kunz WS (1996) Die Zunahme des Flußkontrollkoeffizienten der Cytochrom-C-Oxidase in Kupfer-defizienten Mobr Mäusen. Jahrb. Neuromusk. Erkrankungen 1996, Arcis Verlag: 170-174.
Wisniewski 1995 Eur J Biochem1995Wisniewski E, Gellerich FN, Kunz WS (1995) Distribution of flux control among the enzymes of mitochondrial oxidative phosphorylation in calcium-activated saponin-skinned rat musculus soleus fibers. Eur J Biochem 230:549-54.
Kunz 1995 Biochem Mol Med1995Kunz D, Luley C, Fritz S, Bohnensack R, Winkler K, Kunz WS, Wallesch CW (1995) Oxygraphic evaluation of mitochondrial function in digitonin-permeabilized mononuclear cells and cultured skin fibroblasts of patients with chronic progressive external ophthalmoplegia. Biochem Mol Med 54:105-11.
Winkler 1995 Biochim Biophys Acta1995Winkler K, Kuznetsov AV, Lins H, Kirches E, von Bossanyi P, Dietzmann K, Frank B, Feistner H, Kunz WS (1995) Laser-excited fluorescence studies of mitochondrial function in saponin-skinned skeletal muscle fibers of patients with chronic progressive external ophthalmoplegia. Biochim Biophys Acta 1272:181-4.
Gellerich 1995 HRR1995Gellerich FN, Skladal D, Schranzhofer R, Lanznaster N, Kunz WS, Wisniewski E, Kuznetsov A, Krishmer M, Gnaiger E, Margreiter R, Sperl W (1995) Hochauflösende Respirometrie und multiple Substrat/Inhibitor-Titration zum funktionellen Nachweis mitochondrialer Defekte in permeabilisierten Muskelfasern. In: Molekulare Grundlage hereditärer Myopathien. (Gross M, Gresser U, Hrsg), Zuckschwerdt Verlag, München, Bern, Wien, New York:52-66.
Kuznetsov 1994 BTK-2751994
BTK1994
Kuznetsov AV, Clark JF, Winkler K, Kunz WS (1994) Change in flux control coefficient of cytochrome c oxidase in copper deficient mottled brindled mice. In: What is Controlling Life? (Gnaiger E, Gellerich FN, Wyss M, eds) Modern Trends in BioThermoKinetics 3. Innsbruck Univ Press:275-77.
Kunz 1994 Anal Biochem1994Kunz WS, Kuznetsov AV, Winkler K, Gellerich FN, Neuhof S, Neumann HW (1994) Measurement of fluorescence changes of NAD(P)H and fluorescent flavoproteins in saponin-skinned fibers. Anal Biochem 216:322-7.
Khuchua 1994 Biochim Biophys Acta1994Khuchua ZA, Kuznetsov AV, Schild L, Neumann HW, Kunz WS (1994) The influence of caffeine and Ca2+ on oxidative phosphorylation in saponin-skinned human skeletal muscle fibers. Biochim Biophys Acta 1188:373-9.
Kunz 1993 Biochim Biophys Acta1993Kunz WS, Kuznetsov AV, Schulze W, Eichhorn K, Schild L, Striggow F, Bohnensack R, Grasshoff H, Neumann HW, Gellerich FN (1993) Functional characterization of mitochondrial oxidative phosphorylation in saponin-skinned human muscle fibers. Biochim Biophys Acta 1144:46-53.
Kunz 1993 FEBS Lett1993Kunz WS, Kuznetsov AV, Gellerich FN (1993) Mitochondrial oxidative phosphorylation in saponin-skinned human muscle fibers is stimulated by caffeine. FEBS Lett 323:188-90.

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