ID/Version |
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Sequence description from provider |
RecName: Full=Dynamin-like GTPase OPA1, mitochondrial; EC=3.6.5.5 {ECO:0000269|PubMed:28636943};AltName: Full=Large GTP-binding protein {ECO:0000303|PubMed:11847212}; Short=LargeG {ECO:0000303|PubMed:11847212};AltName: Full=Optic atrophy p | ||||||||||||||
Provider | SWISS-PROT | ||||||||||||||
Sequence |
Polypeptide
960
aa
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Annotated genes and markers |
Follow the symbol links to get more information on the GO terms,
expression assays, orthologs, phenotypic alleles, and other information
for the genes or markers below.
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Sequence references in MGI |
J:76355
Misaka T, et al., Primary structure of a dynamin-related mouse mitochondrial GTPase and its distribution in brain, subcellular localization, and effect on mitochondrial morphology. J Biol Chem. 2002 May 3;277(18):15834-42
J:99680 The FANTOM Consortium and RIKEN Genome Exploration Research Group and Genome Science Group (Genome Network Project Core Group), The Transcriptional Landscape of the Mammalian Genome. Science. 2005;309(5740):1559-1563 J:116016 Frezza C, et al., OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion. Cell. 2006 Jul 14;126(1):177-89 J:116017 Cipolat S, et al., Mitochondrial rhomboid PARL regulates cytochrome c release during apoptosis via OPA1-dependent cristae remodeling. Cell. 2006 Jul 14;126(1):163-75 J:117614 Duvezin-Caubet S, et al., Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology. J Biol Chem. 2006 Dec 8;281(49):37972-9 J:121779 Davies VJ, et al., Opa1 deficiency in a mouse model of autosomal dominant optic atrophy impairs mitochondrial morphology, optic nerve structure and visual function. Hum Mol Genet. 2007 Jun 1;16(11):1307-18 J:154966 Alavi MV, et al., A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy. Brain. 2007 Apr;130(Pt 4):1029-42 J:156874 Ehses S, et al., Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1. J Cell Biol. 2009 Dec 28;187(7):1023-36 J:173140 Darshi M, et al., ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function. J Biol Chem. 2011 Jan 28;286(4):2918-32 J:184716 Quiros PM, et al., Loss of mitochondrial protease OMA1 alters processing of the GTPase OPA1 and causes obesity and defective thermogenesis in mice. EMBO J. 2012 May 2;31(9):2117-33 J:208034 Baker MJ, et al., Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics. EMBO J. 2014 Mar 18;33(6):578-93 J:213150 Mishra P, et al., Proteolytic cleavage of Opa1 stimulates mitochondrial inner membrane fusion and couples fusion to oxidative phosphorylation. Cell Metab. 2014 Apr 1;19(4):630-41 J:214492 Cogliati S, et al., Mitochondrial cristae shape determines respiratory chain supercomplexes assembly and respiratory efficiency. Cell. 2013 Sep 26;155(1):160-71 J:215813 Anand R, et al., The i-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission. J Cell Biol. 2014 Mar 17;204(6):919-29 J:216587 Patten DA, et al., OPA1-dependent cristae modulation is essential for cellular adaptation to metabolic demand. EMBO J. 2014 Nov 18;33(22):2676-91 J:229455 Wai T, et al., Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice. Science. 2015 Dec 4;350(6265):aad0116 J:254859 Huang G, et al., WBSCR16 Is a Guanine Nucleotide Exchange Factor Important for Mitochondrial Fusion. Cell Rep. 2017 Jul 25;20(4):923-934 J:292518 Huttlin EL, et al., A tissue-specific atlas of mouse protein phosphorylation and expression. Cell. 2010 Dec 23;143(7):1174-89 J:309720 Wang R, et al., Identification of new OPA1 cleavage site reveals that short isoforms regulate mitochondrial fusion. Mol Biol Cell. 2021 Jan 15;32(2):157-168 J:340475 Baixauli F, et al., An LKB1-mitochondria axis controls T(H)17 effector function. Nature. 2022 Oct;610(7932):555-561 J:347145 Del Dotto V, et al., OPA1 Isoforms in the Hierarchical Organization of Mitochondrial Functions. Cell Rep. 2017 Jun 20;19(12):2557-2571 J:347149 Murata D, et al., Mitochondrial Safeguard: a stress response that offsets extreme fusion and protects respiratory function via flickering-induced Oma1 activation. EMBO J. 2020 Dec 15;39(24):e105074 J:347299 Fry MY, et al., In situ architecture of Opa1-dependent mitochondrial cristae remodeling. EMBO J. 2024 Feb;43(3):391-413 |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 03/25/2025 MGI 6.24 |
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