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Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion.


ABSTRACT: Mitochondrial morphology changes in response to various stimuli but the significance of this is unclear. In a screen for mutants with abnormal mitochondrial morphology, we identified MMA-1, the Caenorhabditis elegans homolog of the French Canadian Leigh Syndrome protein LRPPRC (leucine-rich pentatricopeptide repeat containing). We demonstrate that reducing mma-1 or LRPPRC function causes mitochondrial hyperfusion. Reducing mma-1/LRPPRC function also decreases the activity of complex IV of the electron transport chain, however without affecting cellular ATP levels. Preventing mitochondrial hyperfusion in mma-1 animals causes larval arrest and embryonic lethality. Furthermore, prolonged LRPPRC knock-down in mammalian cells leads to mitochondrial fragmentation and decreased levels of ATP. These findings indicate that in a mma-1/LRPPRC-deficient background, hyperfusion allows mitochondria to maintain their functions despite a reduction in complex IV activity. Our data reveal an evolutionary conserved mechanism that is triggered by reduced complex IV function and that induces mitochondrial hyperfusion to transiently compensate for a drop in the activity of the electron transport chain.

SUBMITTER: Rolland SG 

PROVIDER: S-EPMC3740885 | biostudies-other | 2013 Aug

REPOSITORIES: biostudies-other

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Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion.

Rolland Stéphane G SG   Motori Elisa E   Memar Nadin N   Hench Jürgen J   Frank Stephan S   Winklhofer Konstanze F KF   Conradt Barbara B  

Proceedings of the National Academy of Sciences of the United States of America 20130722 32


Mitochondrial morphology changes in response to various stimuli but the significance of this is unclear. In a screen for mutants with abnormal mitochondrial morphology, we identified MMA-1, the Caenorhabditis elegans homolog of the French Canadian Leigh Syndrome protein LRPPRC (leucine-rich pentatricopeptide repeat containing). We demonstrate that reducing mma-1 or LRPPRC function causes mitochondrial hyperfusion. Reducing mma-1/LRPPRC function also decreases the activity of complex IV of the el  ...[more]

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