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Sequence Detail
ID/Version
P58281 Q3ULA5 Q8BM08 Q8BKU7 H7BX01 Q8BLL3 Q8R3J7 A6H6Q3 (UniProt | EBI) Last sequence update: 2001-09-26
Last annotation update: 2025-02-05
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
For this sequence
Source
Organism mouse
See UniProt | EBI for source
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.
Type Symbol Name GO Terms Expression
Assays
Orthologs Phenotypic
Alleles
Gene Opa1 OPA1, mitochondrial dynamin like GTPase 126 104 3 23
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

Contributing Projects:
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
The Jackson Laboratory