Zobrazeno 1 - 10
of 15
pro vyhledávání: '"Runu Dey"'
Publikováno v:
Molecular and Cellular Biochemistry. 281:27-33
A calcium-activated protease caldonopain in the cytosolic fraction of Leishmania donovani has been found to digest different endogenous proteins when subjected to SDS-PAGE. Gelatin-embedded gel electrophoresis confirms presence of calcium-dependent p
Publikováno v:
Molecular Genetics and Metabolism. 76:344-347
Mutations in the E3-binding protein component of pyruvate dehydrogenase complex have been demonstrated in a few cases of Leigh syndrome. We report that two mutations previously detected in the E3-binding protein cDNA are the consequence of splice-sit
Autor:
Alessandro Agostino, Cécile Marsac, C Benelli, Isabelle Desguerre, Marie O. Pequignot, Marzia Tartari, Massimo Zeviani, Carina Prip-Buus, Marc Abitbol, Dominique Marchant, Runu Dey, Françoise Fouque
Publikováno v:
Journal of Biological Chemistry. 276:15326-15329
The gene SURF1 encodes a factor involved in the biogenesis of cytochrome c oxidase, the last complex in the respiratory chain. Mutations of the SURF1 gene result in Leigh syndrome and severe cytochrome c oxidase deficiency. Analysis of seven unrelate
Autor:
Carlos T. Moraes, Runu Dey
Publikováno v:
Journal of Biological Chemistry. 275:7087-7094
We explored the role of low mitochondrial membrane potential (DeltaPsim) and the lack of oxidative phosphorylation in apoptosis by assessing the susceptibility of osteosarcoma cell lines with and without mitochondrial DNA to staurosporine-induced dea
Autor:
D. Fontan, Françoise Fouque, I. Redonnet-Vernhet, Runu Dey, Monique Malgat, Cécile Marsac, C Benelli
Publikováno v:
Journal of Inherited Metabolic Disease. 25:325-327
We identified a new Y243S mutation in the X-linked E1 α-PDH gene in a patient with pyruvate dehydrogenase complex (PDHc) deficiency. The activity in cultured fibroblasts was very low even in the presence of high thiamine pyrophosphate (TPP) concentr
Autor:
Abdelhamid Slama, Manuèle Miné, Bernard Aral, Loic Van Den Berghe, Cécile Marsac, Runu Dey, Michèle Brivet, Isabelle Desguerre
Publikováno v:
Annals of neurology. 53(2)
We report a case of neonatal congenital lactic acidosis associated with pyruvate dehydrogenase E3-binding protein deficiency in a newborn girl. She had a severe encephalopathy, and magnetic resonance imaging of the brain showed large subependymal cys
Publikováno v:
Molecular biology and evolution. 17(10)
Respiring mitochondria require many interactions between nuclear and mitochondrial genomes. Although mitochondrial DNA (mtDNA) from the gorilla and the chimpanzee are able to restore oxidative phosphorylation in a human cell, mtDNAs from more distant
Publikováno v:
Neuromuscular disorders : NMD. 10(7)
We report a novel mitochondrial DNA alteration in a 12-year-old boy with myopathy. We identified a single nucleotide insertion (an adenine) in the mitochondrial tRNA-glutamine gene. This addition of an additional adenine in a polyadenine stretch (at
Publikováno v:
The Journal of biological chemistry. 275(40)
The co-evolution of nuclear and mitochondrial genomes in vertebrates led to more than 100 specific interactions that are crucial for an optimized ATP generation. These interactions have been examined by introducing rat mtDNA into mouse cells devoid o