Analysis of the mitochondrial proteome of cybrid cells harbouring a truncative mitochondrial DNA mutation in respiratory complex I

2014 ◽  
Vol 10 (6) ◽  
pp. 1313 ◽  
Author(s):  
Clara Musicco ◽  
Antonella Cormio ◽  
Maria Antonietta Calvaruso ◽  
Luisa Iommarini ◽  
Giuseppe Gasparre ◽  
...  
Keyword(s):  
Mitochondrial Dna ◽  
Complex I ◽  
Mitochondrial Dna Mutation ◽  
Dna Mutation ◽  
Mitochondrial Proteome ◽  
Respiratory Complex ◽  
Respiratory Complex I

scholarly journals Nuclear Suppression of Mitochondrial Defects in Cells without the ND6 Subunit

2006 ◽  
Vol 26 (3) ◽  
pp. 1077-1086 ◽  
Author(s):  
Jian-Hong Deng ◽  
Youfen Li ◽  
Jeong Soon Park ◽  
Jun Wu ◽  
Peiqing Hu ◽  
...  
Keyword(s):  
Mitochondrial Dna ◽  
Complex I ◽  
Mouse Cell ◽  
Mitochondrial Dna Mutation ◽  
Dna Mutation ◽  
Nuclear Background ◽  
Mitochondrial Defects ◽  
Nd5 Gene ◽  
Respiratory Complex I ◽  
Complex I Assembly

ABSTRACT Previously, we characterized a mouse cell line, 4A, carrying a mitochondrial DNA mutation in the subunit for respiratory complex I, NADH dehydrogenase, in the ND6 gene. This mutation abolished the complex I assembly and disrupted the respiratory function of complex I. We now report here that a galactose-resistant clone, 4AR, was isolated from the cells carrying the ND6 mutation. 4AR still contained the homoplasmic mutation, and apparently there was no ND6 protein synthesis, whereas the assembly of other complex I subunits into complex I was recovered. Furthermore, the respiratory activity and mitochondrial membrane potential were fully recovered. To investigate the genetic origin of this compensation, the mitochondrial DNA (mtDNA) from 4AR was transferred to a new nuclear background. The transmitochondrial lines failed to grow in galactose medium. We further transferred mtDNA with a nonsense mutation at the ND5 gene to the 4AR nuclear background, and a suppression for mitochondrial deficiency was observed. Our results suggest that change(s) in the expression of a certain nucleus-encoded factor(s) can compensate for the absence of the ND6 or ND5 subunit.

scholarly journals Nuclear but Not Mitochondrial DNA Involvement in Respiratory Complex I Defects Found in Senescence-Accelerated Mouse Strain, SAMP8

2011 ◽  
Vol 60 (4) ◽  
pp. 397-404 ◽  
Author(s):  
Hirotake IMANISHI ◽  
Mutsumi YOKOTA ◽  
Masayuki MORI ◽  
Akinori SHIMIZU ◽  
Kazuto NAKADA ◽  
...  
Keyword(s):  
Mitochondrial Dna ◽  
Mouse Strain ◽  
Complex I ◽  
Respiratory Complex ◽  
Respiratory Complex I ◽  
Senescence Accelerated Mouse

A novel recurrent mitochondrial DNA mutation inND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia

2007 ◽  
Vol 143A (1) ◽  
pp. 33-41 ◽  
Author(s):  
Emmanuelle Sarzi ◽  
Michael D. Brown ◽  
Sophie Lebon ◽  
Dominique Chretien ◽  
Arnold Munnich ◽  
...  
Keyword(s):  
Mitochondrial Dna ◽  
Complex I ◽  
Leigh Syndrome ◽  
Mitochondrial Dna Mutation ◽  
Dna Mutation ◽  
Complex I Deficiency
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