نتایج جستجو برای: mitochondrial encephalomyopathy

تعداد نتایج: 132426  

Journal: :Human molecular genetics 2009
Sarika Srivastava Francisca Diaz Luisa Iommarini Karine Aure Anne Lombes Carlos T Moraes

Members of the peroxisome proliferator-activated receptor gamma coactivator (PGC) family are potent inducers of mitochondrial biogenesis. We have tested the potential effect of increased mitochondrial biogenesis in cells derived from patients harboring oxidative phosphorylation defects due to either nuclear or mitochondrial DNA mutations. We found that the PGC-1alpha and/or PGC-1beta expression...

2003
Gian Maria Fabrizi Elena Cardaioli Gaetano Salvatore Tiziana Cavallaro Alessandro Malandrini Letizia Manneschi Maria Teresa Dotti Antonio Federico Giancarlo Guazzi

Received 31 October 1995 and in final revised form 19 February 1996 Accepted 23 February 1996 Abstract Objective-To verify the phenotype to genotype correlations of mitochondrial DNA (mtDNA) related disorders in an atypical maternally inherited encephalomyopathy. Methods-Neuroradiological, morphological, biochemical, and molecular genetic analyses were performed on the affected members of a ped...

Journal: :Journal of immunology 2003
Yiping Gu Chunjie Wang Chaim M Roifman Amos Cohen

Mitochondrial DNA is subject to increased rates of mutations due to its proximity to the source of reactive oxygen species. Here we show that increased MHC class I (MHC I) expression serves to alert the immune system to cells with mitochondrial mutations. MHC I is overexpressed in fibroblasts with mitochondrial dysfunction from patients with mitochondrial encephalomyopathy, lactic acidosis, and...

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