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000137010 0247_ $$2doi$$a10.1073/pnas.1303872110
000137010 0247_ $$2pmid$$apmid:23878239
000137010 0247_ $$2pmc$$apmc:PMC3740885
000137010 0247_ $$2ISSN$$a0027-8424
000137010 0247_ $$2ISSN$$a1091-6490
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000137010 037__ $$aDZNE-2020-03332
000137010 041__ $$aEnglish
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000137010 1001_ $$0P:(DE-HGF)0$$aRolland, Stéphane G$$b0$$eCorresponding author
000137010 245__ $$aImpaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion.
000137010 260__ $$aWashington, DC$$bNational Acad. of Sciences$$c2013
000137010 264_1 $$2Crossref$$3online$$bProceedings of the National Academy of Sciences$$c2013-07-22
000137010 264_1 $$2Crossref$$3print$$bProceedings of the National Academy of Sciences$$c2013-08-06
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000137010 520__ $$aMitochondrial morphology changes in response to various stimuli but the significance of this is unclear. In a screen for mutants with abnormal mitochondrial morphology, we identified MMA-1, the Caenorhabditis elegans homolog of the French Canadian Leigh Syndrome protein LRPPRC (leucine-rich pentatricopeptide repeat containing). We demonstrate that reducing mma-1 or LRPPRC function causes mitochondrial hyperfusion. Reducing mma-1/LRPPRC function also decreases the activity of complex IV of the electron transport chain, however without affecting cellular ATP levels. Preventing mitochondrial hyperfusion in mma-1 animals causes larval arrest and embryonic lethality. Furthermore, prolonged LRPPRC knock-down in mammalian cells leads to mitochondrial fragmentation and decreased levels of ATP. These findings indicate that in a mma-1/LRPPRC-deficient background, hyperfusion allows mitochondria to maintain their functions despite a reduction in complex IV activity. Our data reveal an evolutionary conserved mechanism that is triggered by reduced complex IV function and that induces mitochondrial hyperfusion to transiently compensate for a drop in the activity of the electron transport chain.
000137010 536__ $$0G:(DE-HGF)POF3-341$$a341 - Molecular Signaling (POF3-341)$$cPOF3-341$$fPOF III$$x0
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000137010 650_7 $$2NLM Chemicals$$aCaenorhabditis elegans Proteins
000137010 650_7 $$2NLM Chemicals$$aDNA-Binding Proteins
000137010 650_7 $$2NLM Chemicals$$aLRPPRC protein, human
000137010 650_7 $$2NLM Chemicals$$aMMA-1 protein, C elegans
000137010 650_7 $$2NLM Chemicals$$aMembrane Proteins
000137010 650_7 $$2NLM Chemicals$$aMitochondrial Proteins
000137010 650_7 $$2NLM Chemicals$$aNeoplasm Proteins
000137010 650_7 $$2NLM Chemicals$$aSCO1 protein, human
000137010 650_7 $$2NLM Chemicals$$aTFAM protein, human
000137010 650_7 $$2NLM Chemicals$$aTranscription Factors
000137010 650_7 $$0147336-22-9$$2NLM Chemicals$$aGreen Fluorescent Proteins
000137010 650_7 $$08L70Q75FXE$$2NLM Chemicals$$aAdenosine Triphosphate
000137010 650_7 $$0EC 1.9.3.1$$2NLM Chemicals$$aElectron Transport Complex IV
000137010 650_7 $$0EC 3.6.1.-$$2NLM Chemicals$$aGTP Phosphohydrolases
000137010 650_7 $$0EC 3.6.1.-$$2NLM Chemicals$$aOPA1 protein, human
000137010 650_2 $$2MeSH$$aAdenosine Triphosphate: metabolism
000137010 650_2 $$2MeSH$$aAnimals
000137010 650_2 $$2MeSH$$aAnimals, Genetically Modified
000137010 650_2 $$2MeSH$$aBlotting, Western
000137010 650_2 $$2MeSH$$aCaenorhabditis elegans: genetics
000137010 650_2 $$2MeSH$$aCaenorhabditis elegans: metabolism
000137010 650_2 $$2MeSH$$aCaenorhabditis elegans Proteins: genetics
000137010 650_2 $$2MeSH$$aCaenorhabditis elegans Proteins: metabolism
000137010 650_2 $$2MeSH$$aCell Line
000137010 650_2 $$2MeSH$$aCell Line, Tumor
000137010 650_2 $$2MeSH$$aDNA-Binding Proteins: genetics
000137010 650_2 $$2MeSH$$aDNA-Binding Proteins: metabolism
000137010 650_2 $$2MeSH$$aElectron Transport Complex IV: metabolism
000137010 650_2 $$2MeSH$$aGTP Phosphohydrolases: genetics
000137010 650_2 $$2MeSH$$aGTP Phosphohydrolases: metabolism
000137010 650_2 $$2MeSH$$aGreen Fluorescent Proteins: genetics
000137010 650_2 $$2MeSH$$aGreen Fluorescent Proteins: metabolism
000137010 650_2 $$2MeSH$$aHumans
000137010 650_2 $$2MeSH$$aLeigh Disease: genetics
000137010 650_2 $$2MeSH$$aLeigh Disease: metabolism
000137010 650_2 $$2MeSH$$aLeigh Disease: pathology
000137010 650_2 $$2MeSH$$aMembrane Proteins: genetics
000137010 650_2 $$2MeSH$$aMembrane Proteins: metabolism
000137010 650_2 $$2MeSH$$aMicroscopy, Fluorescence
000137010 650_2 $$2MeSH$$aMitochondria: genetics
000137010 650_2 $$2MeSH$$aMitochondria: metabolism
000137010 650_2 $$2MeSH$$aMitochondrial Proteins: genetics
000137010 650_2 $$2MeSH$$aMitochondrial Proteins: metabolism
000137010 650_2 $$2MeSH$$aNeoplasm Proteins: genetics
000137010 650_2 $$2MeSH$$aNeoplasm Proteins: metabolism
000137010 650_2 $$2MeSH$$aRNA Interference
000137010 650_2 $$2MeSH$$aTranscription Factors: genetics
000137010 650_2 $$2MeSH$$aTranscription Factors: metabolism
000137010 7001_ $$aMotori, Elisa$$b1
000137010 7001_ $$aMemar, Nadin$$b2
000137010 7001_ $$aHench, Jürgen$$b3
000137010 7001_ $$aFrank, Stephan$$b4
000137010 7001_ $$0P:(DE-2719)9000369$$aWinklhofer, Konstanze F$$b5$$udzne
000137010 7001_ $$aConradt, Barbara$$b6
000137010 77318 $$2Crossref$$3journal-article$$a10.1073/pnas.1303872110$$b : Proceedings of the National Academy of Sciences, 2013-07-22$$n32$$pE2967-E2976$$tProceedings of the National Academy of Sciences$$v110$$x0027-8424$$y2013
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000137010 8567_ $$2Pubmed Central$$uhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC3740885
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000137010 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)9000369$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b5$$kDZNE
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