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| 001 | 137010 | ||
| 005 | 20240321220156.0 | ||
| 024 | 7 | _ | |a 10.1073/pnas.1303872110 |2 doi |
| 024 | 7 | _ | |a pmid:23878239 |2 pmid |
| 024 | 7 | _ | |a pmc:PMC3740885 |2 pmc |
| 024 | 7 | _ | |a 0027-8424 |2 ISSN |
| 024 | 7 | _ | |a 1091-6490 |2 ISSN |
| 024 | 7 | _ | |a altmetric:1680700 |2 altmetric |
| 037 | _ | _ | |a DZNE-2020-03332 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 500 |
| 100 | 1 | _ | |a Rolland, Stéphane G |0 P:(DE-HGF)0 |b 0 |e Corresponding author |
| 245 | _ | _ | |a Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion. |
| 260 | _ | _ | |a Washington, DC |c 2013 |b National Acad. of Sciences |
| 264 | _ | 1 | |3 online |2 Crossref |b Proceedings of the National Academy of Sciences |c 2013-07-22 |
| 264 | _ | 1 | |3 print |2 Crossref |b Proceedings of the National Academy of Sciences |c 2013-08-06 |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1589477614_7797 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 520 | _ | _ | |a 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. |
| 536 | _ | _ | |a 341 - Molecular Signaling (POF3-341) |0 G:(DE-HGF)POF3-341 |c POF3-341 |f POF III |x 0 |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, |
| 650 | _ | 7 | |a Caenorhabditis elegans Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a DNA-Binding Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a LRPPRC protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a MMA-1 protein, C elegans |2 NLM Chemicals |
| 650 | _ | 7 | |a Membrane Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Mitochondrial Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Neoplasm Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a SCO1 protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a TFAM protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a Transcription Factors |2 NLM Chemicals |
| 650 | _ | 7 | |a Green Fluorescent Proteins |0 147336-22-9 |2 NLM Chemicals |
| 650 | _ | 7 | |a Adenosine Triphosphate |0 8L70Q75FXE |2 NLM Chemicals |
| 650 | _ | 7 | |a Electron Transport Complex IV |0 EC 1.9.3.1 |2 NLM Chemicals |
| 650 | _ | 7 | |a GTP Phosphohydrolases |0 EC 3.6.1.- |2 NLM Chemicals |
| 650 | _ | 7 | |a OPA1 protein, human |0 EC 3.6.1.- |2 NLM Chemicals |
| 650 | _ | 2 | |a Adenosine Triphosphate: metabolism |2 MeSH |
| 650 | _ | 2 | |a Animals |2 MeSH |
| 650 | _ | 2 | |a Animals, Genetically Modified |2 MeSH |
| 650 | _ | 2 | |a Blotting, Western |2 MeSH |
| 650 | _ | 2 | |a Caenorhabditis elegans: genetics |2 MeSH |
| 650 | _ | 2 | |a Caenorhabditis elegans: metabolism |2 MeSH |
| 650 | _ | 2 | |a Caenorhabditis elegans Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Caenorhabditis elegans Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a Cell Line |2 MeSH |
| 650 | _ | 2 | |a Cell Line, Tumor |2 MeSH |
| 650 | _ | 2 | |a DNA-Binding Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a DNA-Binding Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a Electron Transport Complex IV: metabolism |2 MeSH |
| 650 | _ | 2 | |a GTP Phosphohydrolases: genetics |2 MeSH |
| 650 | _ | 2 | |a GTP Phosphohydrolases: metabolism |2 MeSH |
| 650 | _ | 2 | |a Green Fluorescent Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Green Fluorescent Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a Humans |2 MeSH |
| 650 | _ | 2 | |a Leigh Disease: genetics |2 MeSH |
| 650 | _ | 2 | |a Leigh Disease: metabolism |2 MeSH |
| 650 | _ | 2 | |a Leigh Disease: pathology |2 MeSH |
| 650 | _ | 2 | |a Membrane Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Membrane Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a Microscopy, Fluorescence |2 MeSH |
| 650 | _ | 2 | |a Mitochondria: genetics |2 MeSH |
| 650 | _ | 2 | |a Mitochondria: metabolism |2 MeSH |
| 650 | _ | 2 | |a Mitochondrial Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Mitochondrial Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a Neoplasm Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a Neoplasm Proteins: metabolism |2 MeSH |
| 650 | _ | 2 | |a RNA Interference |2 MeSH |
| 650 | _ | 2 | |a Transcription Factors: genetics |2 MeSH |
| 650 | _ | 2 | |a Transcription Factors: metabolism |2 MeSH |
| 700 | 1 | _ | |a Motori, Elisa |b 1 |
| 700 | 1 | _ | |a Memar, Nadin |b 2 |
| 700 | 1 | _ | |a Hench, Jürgen |b 3 |
| 700 | 1 | _ | |a Frank, Stephan |b 4 |
| 700 | 1 | _ | |a Winklhofer, Konstanze F |0 P:(DE-2719)9000369 |b 5 |u dzne |
| 700 | 1 | _ | |a Conradt, Barbara |b 6 |
| 773 | 1 | 8 | |a 10.1073/pnas.1303872110 |b : Proceedings of the National Academy of Sciences, 2013-07-22 |n 32 |p E2967-E2976 |3 journal-article |2 Crossref |t Proceedings of the National Academy of Sciences |v 110 |y 2013 |x 0027-8424 |
| 773 | _ | _ | |a 10.1073/pnas.1303872110 |g Vol. 110, no. 32, p. E2967 - E2976 |0 PERI:(DE-600)1461794-8 |n 32 |q 110:32 |t Proceedings of the National Academy of Sciences of the United States of America |v 110 |y 2013 |x 0027-8424 |
| 856 | 7 | _ | |2 Pubmed Central |u http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3740885 |
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| 914 | 1 | _ | |y 2013 |
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