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Difference between revisions of "Iyer 2012 Hum Gene Ther"

From Bioblast
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{{Labeling
{{Labeling
|area=mt-Biogenesis; mt-density
|area=Respiration, mt-Biogenesis; mt-density, Genetic knockout; overexpression, Mitochondrial medicine
|organism=Human
|organism=Human
|model cell lines=Fibroblast
|model cell lines=Fibroblast
|preparations=Intact cells
|preparations=Intact cells
|injuries=Mitochondrial Disease; Degenerative Disease and Defect, Genetic Defect; Knockdown; Overexpression
|diseases=Inborn mt-disease
|topics=mt-Biogenesis; mt-density
|couplingstates=OXPHOS
|couplingstates=OXPHOS
|instruments=Oxygraph-2k
|instruments=Oxygraph-2k
}}
}}
* [http://www.news.vcu.edu/news/Researchers_Study_and_Develop_Approach_to_Treat_Mitochondrial Highlights on the VCU-News: Researchers Study and Develop Approach to Treat Mitochondrial Disorders].
* [http://www.news.vcu.edu/news/Researchers_Study_and_Develop_Approach_to_Treat_Mitochondrial Highlights on the VCU-News: Researchers Study and Develop Approach to Treat Mitochondrial Disorders].

Revision as of 16:41, 10 August 2013

Publications in the MiPMap
Iyer 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: 647-657.

» PMID: 22390282

Iyer S, Bergquist K, Young K, Gnaiger E, Rao RR, Bennett JP (2012) Hum Gene Ther

Abstract: Many incurable mitochondrial disorders result from mutant mitochondrial DNA (mtDNA) and impaired respiration. Leigh’s syndrome (LS) is a fatal neurodegenerative disorder of infants and Leber’s hereditary optic neuropathy (LHON) causes blindness in young adults. Treatment of LHON and LS cells respectively harboring G11778A and T8993G mutant mtDNA by >90%, with healthy donor mtDNA complexed with recombinant human mitochondrial transcription factor A (rhTFAM), improved mitochondrial respiration by ~1.2 fold in LHON cells and restored ~>50% ATP synthase function in LS cells. Mitochondrial replication, transcription and translation of key respiratory genes and proteins were increased in the short term. Increased NRF1, TFAMB1 and TFAMA expression alluded to the activation of mitochondrial biogenesis as a mechanism for improving mitochondrial respiration. These results represent the development of a therapeutic approach for LHON and LS patients in the near future. Keywords: TFAM, mtDNA, Leigh’s syndrome, LHON disease, mitochondrial respiration, biogenesis, transcription, diseased fibroblast, cybrid cells

O2k-Network Lab: US VA Richmond Iyer S, US VA Richmond Bennett JP, AT Innsbruck Gnaiger E, AT Innsbruck MitoCom, US VA Richmond Virginia Commonwealth Univ


Labels: MiParea: Respiration, mt-Biogenesis; mt-density"mt-Biogenesis; mt-density" is not in the list (Respiration, Instruments;methods, mt-Biogenesis;mt-density, mt-Structure;fission;fusion, mt-Membrane, mtDNA;mt-genetics, nDNA;cell genetics, Genetic knockout;overexpression, Comparative MiP;environmental MiP, Gender, ...) of allowed values for the "MiP area" property., Genetic knockout; overexpression"Genetic knockout; overexpression" is not in the list (Respiration, Instruments;methods, mt-Biogenesis;mt-density, mt-Structure;fission;fusion, mt-Membrane, mtDNA;mt-genetics, nDNA;cell genetics, Genetic knockout;overexpression, Comparative MiP;environmental MiP, Gender, ...) of allowed values for the "MiP area" property., Mitochondrial medicine"Mitochondrial medicine" is not in the list (Respiration, Instruments;methods, mt-Biogenesis;mt-density, mt-Structure;fission;fusion, mt-Membrane, mtDNA;mt-genetics, nDNA;cell genetics, Genetic knockout;overexpression, Comparative MiP;environmental MiP, Gender, ...) of allowed values for the "MiP area" property.  Pathology: Inborn mt-disease"Inborn mt-disease" is not in the list (Aging;senescence, Alzheimer's, Autism, Cancer, Cardiovascular, COPD, Diabetes, Inherited, Infectious, Myopathy, ...) of allowed values for the "Diseases" property. 

Organism: Human 

Preparation: Intact cells 


Coupling state: OXPHOS 

HRR: Oxygraph-2k