Gene Symbol: Opa1
Description: OPA1, mitochondrial dynamin like GTPase
Alias: 1200011N24Rik, AI225888, AI847218, lilr3, mKIAA0567, dynamin-like 120 kDa protein, mitochondrial, large GTP-binding protein, largeG, optic atrophy 1 homolog, optic atrophy protein 1 homolog
Species: mouse
Products:     Opa1

Top Publications

  1. Misaka T, Miyashita T, Kubo Y. 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;277:15834-42 pubmed
    A new member of the dynamin GTPase family (OPA1) was recently identified in humans and shown to be mutated in patients with dominant optic atrophy...
  2. Piquereau J, Caffin F, Novotova M, Prola A, Garnier A, Mateo P, et al. Down-regulation of OPA1 alters mouse mitochondrial morphology, PTP function, and cardiac adaptation to pressure overload. Cardiovasc Res. 2012;94:408-17 pubmed publisher
    The optic atrophy 1 (OPA1) protein is an essential protein involved in the fusion of the mitochondrial inner membrane. Despite its high level of expression, the role of OPA1 in the heart is largely unknown...
  3. Baker M, Lampe P, Stojanovski D, Korwitz A, Anand R, Tatsuta T, et al. Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics. EMBO J. 2014;33:578-93 pubmed publisher
    ..The OMA1 peptidase mediates the degradation of long isoforms of the dynamin-like GTPase OPA1 in the inner membrane...
  4. Twig G, Elorza A, Molina A, Mohamed H, Wikstrom J, Walzer G, et al. Fission and selective fusion govern mitochondrial segregation and elimination by autophagy. EMBO J. 2008;27:433-46 pubmed publisher
    ..of non-fusing mitochondria that were found to have reduced delta psi(m) and decreased levels of the fusion protein OPA1. Inhibition of the fission machinery through DRP1(K38A) or FIS1 RNAi decreased mitochondrial autophagy and resulted ..
  5. Barnard A, Charbel Issa P, Perganta G, Williams P, Davies V, Sekaran S, et al. Specific deficits in visual electrophysiology in a mouse model of dominant optic atrophy. Exp Eye Res. 2011;93:771-7 pubmed publisher
    Autosomal dominant optic atrophy (ADOA) is a slowly progressive optic neuropathy caused by mutations in the OPA1 gene. OPA1 is ubiquitously expressed and plays a key role in mitochondrial fusion...
  6. Ban T, Heymann J, Song Z, Hinshaw J, Chan D. OPA1 disease alleles causing dominant optic atrophy have defects in cardiolipin-stimulated GTP hydrolysis and membrane tubulation. Hum Mol Genet. 2010;19:2113-22 pubmed publisher
    The dynamin-related GTPase OPA1 is mutated in autosomal dominant optic atrophy (DOA) (Kjer type), an inherited neuropathy of the retinal ganglion cells...
  7. Akepati V, Müller E, Otto A, Strauss H, Portwich M, Alexander C. Characterization of OPA1 isoforms isolated from mouse tissues. J Neurochem. 2008;106:372-83 pubmed publisher
    b>OPA1, a nuclear encoded mitochondrial protein causing autosomal dominant optic atrophy, is a key player in mitochondrial fusion and cristae morphology regulation...
  8. Ishihara N, Nomura M, Jofuku A, Kato H, Suzuki S, Masuda K, et al. Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice. Nat Cell Biol. 2009;11:958-66 pubmed publisher
  9. Nguyen D, Alavi M, Kim K, Kang T, Scott R, Noh Y, et al. A new vicious cycle involving glutamate excitotoxicity, oxidative stress and mitochondrial dynamics. Cell Death Dis. 2011;2:e240 pubmed publisher
    ..Optic atrophy gene 1 (OPA1) is an inner membrane protein important for mitochondrial fusion...

More Information


  1. Chen L, Liu T, Tran A, Lu X, Tomilov A, Davies V, et al. OPA1 mutation and late-onset cardiomyopathy: mitochondrial dysfunction and mtDNA instability. J Am Heart Assoc. 2012;1:e003012 pubmed publisher
    ..Previously we reported that the fusion protein optic atrophy 1 (OPA1) is decreased in heart failure...
  2. Davies V, Hollins A, Piechota M, Yip W, Davies J, White K, 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;16:1307-18 pubmed
    b>OPA1 is a ubiquitously expressed, nuclear dynamin-related GTPase, targeted to the inner mitochondrial membrane, which plays a role in mitochondrial fusion...
  3. Aijaz S, Erskine L, Jeffery G, Bhattacharya S, Votruba M. Developmental expression profile of the optic atrophy gene product: OPA1 is not localized exclusively in the mammalian retinal ganglion cell layer. Invest Ophthalmol Vis Sci. 2004;45:1667-73 pubmed
    ..The OPA1 gene encodes a 960-amino-acid protein...
  4. Yu Wai Man P, Davies V, Piechota M, Cree L, Votruba M, Chinnery P. Secondary mtDNA defects do not cause optic nerve dysfunction in a mouse model of dominant optic atrophy. Invest Ophthalmol Vis Sci. 2009;50:4561-6 pubmed publisher
    The majority of patients with autosomal dominant optic atrophy (DOA) harbor pathogenic OPA1 mutations and certain missense mutations, mostly within the GTPase domain, have recently been shown to cause multiple mitochondrial DNA (mtDNA) ..
  5. Darshi M, Mendiola V, Mackey M, Murphy A, Koller A, Perkins G, et al. ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function. J Biol Chem. 2011;286:2918-32 pubmed publisher
    ..RNAi knockdown of ChChd3 in HeLa cells resulted in fragmented mitochondria, reduced OPA1 protein levels and impaired fusion, and clustering of the mitochondria around the nucleus along with reduced growth ..
  6. Cipolat S, Martins de Brito O, Dal Zilio B, Scorrano L. OPA1 requires mitofusin 1 to promote mitochondrial fusion. Proc Natl Acad Sci U S A. 2004;101:15927-32 pubmed
    ..Mechanisms of mitochondrial fusion are largely uncharacterized in mammalian cells. It is unclear whether OPA1, a dynamin-related protein of the inner membrane mutated in autosomal dominant optic atrophy, participates in ..
  7. Tondera D, Grandemange S, Jourdain A, Karbowski M, Mattenberger Y, Herzig S, et al. SLP-2 is required for stress-induced mitochondrial hyperfusion. EMBO J. 2009;28:1589-600 pubmed publisher
    ..fusion relies on dynamin-related GTPases, the mitofusins (MFN1 and 2) in the outer mitochondrial membrane and OPA1 (optic atrophy 1) in the inner mitochondrial membrane...
  8. Williams P, Piechota M, Von Ruhland C, Taylor E, Morgan J, Votruba M. Opa1 is essential for retinal ganglion cell synaptic architecture and connectivity. Brain. 2012;135:493-505 pubmed publisher
    Retinal ganglion cell dendritic pruning has been reported in association with a 50% reduction in Opa1 transcript and protein in retinal and neural tissue, which manifests as visual dysfunction in the heterozygous mutant mouse, B6;C3-Opa1(..
  9. Williams P, Morgan J, Votruba M. Opa1 deficiency in a mouse model of dominant optic atrophy leads to retinal ganglion cell dendropathy. Brain. 2010;133:2942-51 pubmed publisher
    The heterozygous mutation B6;C3-Opa1(Q285STOP), which models autosomal dominant optic atrophy, leads to a 50% reduction in Opa1 transcript and protein in the mouse retina and neural tissues and is associated with visual dysfunction and ..
  10. Kieper N, Holmström K, Ciceri D, Fiesel F, Wolburg H, Ziviani E, et al. Modulation of mitochondrial function and morphology by interaction of Omi/HtrA2 with the mitochondrial fusion factor OPA1. Exp Cell Res. 2010;316:1213-24 pubmed publisher
    ..the levels of proteins involved in mitochondrial fusion, we found a selective up-regulation of more soluble OPA1 protein...
  11. Song Z, Ghochani M, McCaffery J, Frey T, Chan D. Mitofusins and OPA1 mediate sequential steps in mitochondrial membrane fusion. Mol Biol Cell. 2009;20:3525-32 pubmed publisher
    Mitochondrial fusion requires the coordinated fusion of the outer and inner membranes. Three large GTPases--OPA1 and the mitofusins Mfn1 and Mfn2--are essential for the fusion of mammalian mitochondria...
  12. Quirós P, Ramsay A, Sala D, Fernandez Vizarra E, Rodriguez F, Peinado J, et al. Loss of mitochondrial protease OMA1 alters processing of the GTPase OPA1 and causes obesity and defective thermogenesis in mice. EMBO J. 2012;31:2117-33 pubmed publisher
    ..metalloprotease OMA1 plays an essential role in the proteolytic inactivation of the dynamin-related GTPase OPA1 (optic atrophy 1)...
  13. Alavi M, Fuhrmann N, Nguyen H, Yu Wai Man P, Heiduschka P, Chinnery P, et al. Subtle neurological and metabolic abnormalities in an Opa1 mouse model of autosomal dominant optic atrophy. Exp Neurol. 2009;220:404-9 pubmed publisher
    The ubiquitously expressed gene OPA1 is the main disease causing gene for autosomal dominant optic atrophy (ADOA)...
  14. Wasilewski M, Semenzato M, Rafelski S, Robbins J, Bakardjiev A, Scorrano L. Optic atrophy 1-dependent mitochondrial remodeling controls steroidogenesis in trophoblasts. Curr Biol. 2012;22:1228-34 pubmed publisher
    ..Here we show that the mitochondria-shaping protein Optic atrophy 1 (Opa1) controls efficiency of steroidogenesis...
  15. Sarzi E, Angebault C, Seveno M, Gueguen N, Chaix B, Bielicki G, et al. The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse. Brain. 2012;135:3599-613 pubmed publisher
    ..optic atrophy is a rare inherited optic nerve degeneration caused by mutations in the mitochondrial fusion gene OPA1. Recently, the clinical spectrum of dominant optic atrophy has been extended to frequent syndromic forms, ..
  16. Pesch U, Fries J, Bette S, Kalbacher H, Wissinger B, Alexander C, et al. OPA1, the disease gene for autosomal dominant optic atrophy, is specifically expressed in ganglion cells and intrinsic neurons of the retina. Invest Ophthalmol Vis Sci. 2004;45:4217-25 pubmed
    ..hereditary disorder characterized by progressive loss of vision and caused by mutations in a dynamin-related gene, OPA1, which translates into a protein with a mitochondrial leader sequence...
  17. Anand R, Wai T, Baker M, Kladt N, Schauss A, Rugarli E, et al. The i-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission. J Cell Biol. 2014;204:919-29 pubmed publisher
    Mitochondrial fusion and structure depend on the dynamin-like GTPase OPA1, whose activity is regulated by proteolytic processing...
  18. Dayanithi G, Chen Kuo Chang M, Viero C, Hamel C, Muller A, Lenaers G. Characterization of Ca2+ signalling in postnatal mouse retinal ganglion cells: involvement of OPA1 in Ca2+ clearance. Ophthalmic Genet. 2010;31:53-65 pubmed publisher
    ..signals of RGCs in response to varied drugs with Fura-2 imaging, and secondly tested the role of optic atrophy 1 or OPA1, the gene responsible for Autosomal Dominant Optic Atrophy, on mitochondrial ability to capture intracellular Ca(2+)..
  19. Jahani Asl A, Pilon Larose K, Xu W, Maclaurin J, Park D, McBride H, et al. The mitochondrial inner membrane GTPase, optic atrophy 1 (Opa1), restores mitochondrial morphology and promotes neuronal survival following excitotoxicity. J Biol Chem. 2011;286:4772-82 pubmed publisher
    ..Up-regulation of the mitochondrial inner membrane GTPase, Opa1, is able to restore mitochondrial morphology and protect neurons against excitotoxic injury...
  20. Duvezin Caubet S, Jagasia R, Wagener J, Hofmann S, Trifunovic A, Hansson A, et al. Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology. J Biol Chem. 2006;281:37972-9 pubmed
    ..Mutations in the gene encoding OPA1, a protein required for fusion of mitochondria, are associated with hereditary autosomal dominant optic atrophy ..
  21. Müller Rischart A, Pilsl A, Beaudette P, Patra M, Hadian K, Funke M, et al. The E3 ligase parkin maintains mitochondrial integrity by increasing linear ubiquitination of NEMO. Mol Cell. 2013;49:908-21 pubmed publisher
    ..As a result, the mitochondrial guanosine triphosphatase OPA1 is transcriptionally upregulated via NF-?B-responsive promoter elements for maintenance of mitochondrial integrity ..
  22. Caffin F, Prola A, Piquereau J, Novotova M, David D, Garnier A, et al. Altered skeletal muscle mitochondrial biogenesis but improved endurance capacity in trained OPA1-deficient mice. J Physiol. 2013;591:6017-37 pubmed publisher
    The role of OPA1, a GTPase dynamin protein mainly involved in the fusion of inner mitochondrial membranes, has been studied in many cell types, but only a few studies have been conducted on adult differentiated tissues such as cardiac or ..
  23. Ju W, Kim K, Duong Polk K, Lindsey J, Ellisman M, Weinreb R. Increased optic atrophy type 1 expression protects retinal ganglion cells in a mouse model of glaucoma. Mol Vis. 2010;16:1331-42 pubmed
    The goal of this study is to determine whether increased optic atrophy type 1 (OPA1) expression protects against retinal ganglion cell (RGC) death in glaucomatous DBA/2J mice...
  24. White K, Davies V, Hogan V, Piechota M, Nichols P, Turnbull D, et al. OPA1 deficiency associated with increased autophagy in retinal ganglion cells in a murine model of dominant optic atrophy. Invest Ophthalmol Vis Sci. 2009;50:2567-71 pubmed publisher
    ..RGC) and axonal abnormalities in an ENU-induced mutant mouse carrying a protein-truncating nonsense mutation in OPA1. Mutations in the OPA1 gene cause autosomal dominant optic atrophy (ADOA) in which loss of RGCs followed by myelin ..
  25. Zhang Z, Wakabayashi N, Wakabayashi J, Tamura Y, Song W, Sereda S, et al. The dynamin-related GTPase Opa1 is required for glucose-stimulated ATP production in pancreatic beta cells. Mol Biol Cell. 2011;22:2235-45 pubmed publisher
    Previous studies using in vitro cell culture systems have shown the role of the dynamin-related GTPase Opa1 in apoptosis prevention and mitochondrial DNA (mtDNA) maintenance...
  26. Sanjuán Szklarz L, Scorrano L. The antiapoptotic OPA1/Parl couple participates in mitochondrial adaptation to heat shock. Biochim Biophys Acta. 2012;1817:1886-93 pubmed publisher
    The mitochondria-shaping protein optic atrophy 1 (OPA1) has genetically distinguishable roles in mitochondrial morphology and apoptosis...
  27. Cipolat S, Rudka T, Hartmann D, Costa V, Serneels L, Craessaerts K, et al. Mitochondrial rhomboid PARL regulates cytochrome c release during apoptosis via OPA1-dependent cristae remodeling. Cell. 2006;126:163-75 pubmed
    ..but are no longer protected against intrinsic apoptotic death stimuli by the dynamin-related mitochondrial protein OPA1. Parl-/- mitochondria display reduced levels of a soluble, intermembrane space (IMS) form of OPA1, and OPA1 ..
  28. Alavi M, Bette S, Schimpf S, Schuettauf F, Schraermeyer U, Wehrl H, et al. A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy. Brain. 2007;130:1029-42 pubmed
    ..adOA is most frequently associated with mutations in OPA1 encoding a dynamin-related large GTPase that localizes to mitochondria...
  29. Ehses S, Raschke I, Mancuso G, Bernacchia A, Geimer S, Tondera D, et al. Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1. J Cell Biol. 2009;187:1023-36 pubmed publisher
    Mitochondrial fusion depends on the dynamin-like guanosine triphosphatase OPA1, whose activity is controlled by proteolytic cleavage...
  30. Duvezin Caubet S, Koppen M, Wagener J, Zick M, Israel L, Bernacchia A, et al. OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria. Mol Biol Cell. 2007;18:3582-90 pubmed
    The morphology of mitochondria in mammalian cells is regulated by proteolytic cleavage of OPA1, a dynamin-like GTPase of the mitochondrial inner membrane...
  31. Ju W, Kim K, Lindsey J, Angert M, Duong Polk K, Scott R, et al. Intraocular pressure elevation induces mitochondrial fission and triggers OPA1 release in glaucomatous optic nerve. Invest Ophthalmol Vis Sci. 2008;49:4903-11 pubmed publisher
    ..pressure (IOP) triggers mitochondrial fission and ultrastructural changes and alters optic atrophy type 1 (OPA1) expression and distribution in the optic nerve (ON) of glaucomatous DBA/2J mice...
  32. Heiduschka P, Schnichels S, Fuhrmann N, Hofmeister S, Schraermeyer U, Wissinger B, et al. Electrophysiological and histologic assessment of retinal ganglion cell fate in a mouse model for OPA1-associated autosomal dominant optic atrophy. Invest Ophthalmol Vis Sci. 2010;51:1424-31 pubmed publisher
    ..The main disease-causing gene is OPA1. Here, we examine a mouse carrying a pathogenic mutation in Opa1 by electrophysiological measurements and assess ..
  33. Cogliati S, Frezza C, Soriano M, Varanita T, Quintana Cabrera R, Corrado M, et al. Mitochondrial cristae shape determines respiratory chain supercomplexes assembly and respiratory efficiency. Cell. 2013;155:160-71 pubmed publisher
    ..We demonstrate that, accordingly, the efficiency of mitochondria-dependent cell growth depends on cristae shape. Thus, RCS assembly emerges as a link between membrane morphology and function. ..
  34. Frezza C, Cipolat S, Martins de Brito O, Micaroni M, Beznoussenko G, Rudka T, et al. OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion. Cell. 2006;126:177-89 pubmed
    ..Here we provide evidence that Optic Atrophy 1 (OPA1), a profusion dynamin-related protein of the inner mitochondrial membrane mutated in dominant optic atrophy, ..
  35. Chen H, Chomyn A, Chan D. Disruption of fusion results in mitochondrial heterogeneity and dysfunction. J Biol Chem. 2005;280:26185-92 pubmed
    ..Disruption of OPA1 by RNAi also blocked all mitochondrial fusion and resulted in similar cellular defects...
  36. Yamaguchi R, Lartigue L, Perkins G, Scott R, Dixit A, Kushnareva Y, et al. Opa1-mediated cristae opening is Bax/Bak and BH3 dependent, required for apoptosis, and independent of Bak oligomerization. Mol Cell. 2008;31:557-69 pubmed publisher
    ..full cytochrome c release but only a subtle alteration of crista junctions, which involved the disassembly of Opa1 complexes...
  37. Nan J, Hu H, Sun Y, Zhu L, Wang Y, Zhong Z, et al. TNFR2 Stimulation Promotes Mitochondrial Fusion via Stat3- and NF-kB-Dependent Activation of OPA1 Expression. Circ Res. 2017;121:392-410 pubmed publisher
    ..Importantly, TNFR2 activation-induced increases in OPA1 (optic atrophy 1) protein expression were responsible for the above enhancements, and these changes could be ..
  38. Lee H, Yoon Y. Transient contraction of mitochondria induces depolarization through the inner membrane dynamin OPA1 protein. J Biol Chem. 2014;289:11862-72 pubmed publisher
    ..Furthermore, we discovered that silencing the inner membrane-associated dynamin optic atrophy 1 (OPA1) in fission deficiency prevented mitochondrial depolarization and decreased proton leak without blocking ..
  39. Ryu S, Han E, Yoon J, Choi C. The mitochondrial fusion-related proteins Mfn2 and OPA1 are transcriptionally induced during differentiation of bone marrow progenitors to immature dendritic cells. Mol Cells. 2015;38:89-94 pubmed publisher
    ..Mfn2 and OPA1, but not Mfn1, are transcriptionally upregulated during differentiation; however, knockdown of Mfn2 and OPA1 does ..
  40. Delettre C, Lenaers G, Belenguer P, Hamel C. Gene structure and chromosomal localization of mouse Opa1 : its exclusion from the Bst locus. BMC Genet. 2003;4:8 pubmed
    ..We have previously identified the OPA1 gene and shown that it was mutated in patients with DOA...
  41. Del Dotto V, Mishra P, Vidoni S, Fogazza M, Maresca A, Caporali L, et al. OPA1 Isoforms in the Hierarchical Organization of Mitochondrial Functions. Cell Rep. 2017;19:2557-2571 pubmed publisher
    b>OPA1 is a GTPase that controls mitochondrial fusion, cristae integrity, and mtDNA maintenance...
  42. Varanita T, Soriano M, Romanello V, Zaglia T, Quintana Cabrera R, Semenzato M, et al. The OPA1-dependent mitochondrial cristae remodeling pathway controls atrophic, apoptotic, and ischemic tissue damage. Cell Metab. 2015;21:834-44 pubmed publisher
    ..Here we investigate the role of the optic atrophy 1 (OPA1)-dependent cristae remodeling pathway in vivo and provide evidence that it regulates the response of multiple ..
  43. Sood A, Jeyaraju D, Prudent J, Caron A, Lemieux P, McBride H, et al. A Mitofusin-2-dependent inactivating cleavage of Opa1 links changes in mitochondria cristae and ER contacts in the postprandial liver. Proc Natl Acad Sci U S A. 2014;111:16017-22 pubmed publisher
    ..with the transient expression of two previously unidentified C-terminal fragments (CTFs) of Optic atrophy 1 (Opa1), a mitochondrial GTPase that regulates cristae biogenesis and mitochondria dynamics...
  44. Karbowski M, Norris K, Cleland M, Jeong S, Youle R. Role of Bax and Bak in mitochondrial morphogenesis. Nature. 2006;443:658-62 pubmed
    ..Our results show that Bax and Bak regulate mitochondrial dynamics in healthy cells and indicate that Bcl-2 family members may also regulate apoptosis through organelle morphogenesis machineries. ..
  45. Fülöp L, Rajki A, Katona D, Szanda G, Spät A. Extramitochondrial OPA1 and adrenocortical function. Mol Cell Endocrinol. 2013;381:70-9 pubmed publisher
    We have previously described that silencing of the mitochondrial protein OPA1 enhances mitochondrial Ca(2+) signaling and aldosterone production in H295R adrenocortical cells...
  46. Moore B, Gonzalez Aviles G, Larkins C, Hillman M, Caspary T. Mitochondrial retention of Opa1 is required for mouse embryogenesis. Mamm Genome. 2010;21:350-60 pubmed publisher
    ..Here we demonstrate that the lilR3 mutant mouse phenotype is due to a point mutation in the Opa1 gene resulting in mislocalized Opa1 protein from the ..
  47. Korwitz A, Merkwirth C, Richter Dennerlein R, Tröder S, Sprenger H, Quirós P, et al. Loss of OMA1 delays neurodegeneration by preventing stress-induced OPA1 processing in mitochondria. J Cell Biol. 2016;212:157-66 pubmed publisher
    Proteolytic cleavage of the dynamin-like guanosine triphosphatase OPA1 in mitochondria is emerging as a central regulatory hub that determines mitochondrial morphology under stress and in disease...
  48. Pidoux G, Witczak O, Jarnæss E, Myrvold L, Urlaub H, Stokka A, et al. Optic atrophy 1 is an A-kinase anchoring protein on lipid droplets that mediates adrenergic control of lipolysis. EMBO J. 2011;30:4371-86 pubmed publisher
    ..Here, we report a new function for optic atrophy 1 (OPA1), a protein known to regulate mitochondrial dynamics, as a dual-specificity A-kinase anchoring protein associated ..
  49. Glytsou C, Calvo E, Cogliati S, Mehrotra A, Anastasia I, Rigoni G, et al. Optic Atrophy 1 Is Epistatic to the Core MICOS Component MIC60 in Mitochondrial Cristae Shape Control. Cell Rep. 2016;17:3024-3034 pubmed publisher
    The mitochondrial contact site and cristae organizing system (MICOS) and Optic atrophy 1 (OPA1) control cristae shape, thus affecting mitochondrial function and apoptosis...
  50. Laforge M, Rodrigues V, Silvestre R, Gautier C, Weil R, Corti O, et al. NF-κB pathway controls mitochondrial dynamics. Cell Death Differ. 2016;23:89-98 pubmed publisher
    The Optic atrophy 1 protein (OPA1) is a key element in the dynamics and morphology of mitochondria...
  51. Wang X, Li H, Zheng A, Yang L, Chen C, Tang Y, et al. Mitochondrial dysfunction-associated OPA1 cleavage contributes to muscle degeneration: preventative effect of hydroxytyrosol acetate. Cell Death Dis. 2014;5:e1521 pubmed publisher
    ..Meanwhile, we found that both t-BHP and FCCP promoted the cleavage of optic atrophy 1 (OPA1) from the long form into short form during the early stages...
  52. Rochais F, Dandonneau M, Mesbah K, Jarry T, Mattei M, Kelly R. Hes1 is expressed in the second heart field and is required for outflow tract development. PLoS ONE. 2009;4:e6267 pubmed publisher
    ..Hes1 is expressed in cardiac progenitor cells in the early embryo and is required for development of the arterial pole of the heart. ..
  53. Moulis M, Millet A, Daloyau M, Miquel M, Ronsin B, Wissinger B, et al. OPA1 haploinsufficiency induces a BNIP3-dependent decrease in mitophagy in neurons: relevance to Dominant Optic Atrophy. J Neurochem. 2017;140:485-494 pubmed publisher
    Dominant optic atrophy (DOA) is because of mutations in the mitochondrial protein OPA1. The disease principally affects retinal ganglion cells, whose axons degenerate leading to vision impairments, and sometimes other neuronal phenotypes...
  54. Belenguer P, Pellegrini L. The dynamin GTPase OPA1: more than mitochondria?. Biochim Biophys Acta. 2013;1833:176-83 pubmed publisher
    ..One of them is the mammalian OPA1 (Optic atrophy 1) protein, which resides inside the mitochondrion anchored to the inner membrane and, in a cleaved ..
  55. Patten D, Wong J, Khacho M, Soubannier V, Mailloux R, Pilon Larose K, et al. OPA1-dependent cristae modulation is essential for cellular adaptation to metabolic demand. EMBO J. 2014;33:2676-91 pubmed publisher
    ..Optic atrophy 1 (OPA1) is the mitochondrial GTPase responsible for inner membrane fusion and maintenance of cristae structure...
  56. Ganapathy P, Perry R, Tawfik A, Smith R, Perry E, Roon P, et al. Homocysteine-mediated modulation of mitochondrial dynamics in retinal ganglion cells. Invest Ophthalmol Vis Sci. 2011;52:5551-8 pubmed publisher
    ..Gene and protein expression analyses of Opa1 and Fis1 were performed on cbs?/? neural retinas...
  57. Smith T, Seto S, Ganne P, Votruba M. A randomized, placebo-controlled trial of the benzoquinone idebenone in a mouse model of OPA1-related dominant optic atrophy reveals a limited therapeutic effect on retinal ganglion cell dendropathy and visual function. Neuroscience. 2016;319:92-106 pubmed publisher
    Dominant optic atrophy (DOA) arises from mutations in the OPA1 gene that promotes fusion of the inner mitochondrial membrane and plays a role in maintaining ATP levels...
  58. Patterson V, Zullo A, Koenig C, Stoessel S, Jo H, Liu X, et al. Neural-specific deletion of Htra2 causes cerebellar neurodegeneration and defective processing of mitochondrial OPA1. PLoS ONE. 2014;9:e115789 pubmed publisher
    ..Furthermore, the onset of these structural anomalies was accompanied by defective processing of OPA1, a key molecule for mitochondrial fusion and cristae remodeling, leading to depletion of the L-isoform...
  59. Xiao X, Hu Y, Quirós P, Wei Q, Lopez Otin C, Dong Z. OMA1 mediates OPA1 proteolysis and mitochondrial fragmentation in experimental models of ischemic kidney injury. Am J Physiol Renal Physiol. 2014;306:F1318-26 pubmed publisher
    ..OMA1-mediated proteolysis of OPA1, a key inner membrane fusion protein, was recently suggested to account for inner membrane cleavage during cell ..
  60. Sarzi E, Seveno M, Angebault C, Milea D, Rönnbäck C, Quiles M, et al. Increased steroidogenesis promotes early-onset and severe vision loss in females with OPA1 dominant optic atrophy. Hum Mol Genet. 2016;25:2539-2551 pubmed
    b>OPA1 mutations are responsible for autosomal dominant optic atrophy (ADOA), a progressive blinding disease characterized by retinal ganglion cell (RGC) degeneration and large phenotypic variations, the underlying mechanisms of which are ..
  61. Le Page S, Niro M, Fauconnier J, Cellier L, Tamareille S, Gharib A, et al. Increase in Cardiac Ischemia-Reperfusion Injuries in Opa1+/- Mouse Model. PLoS ONE. 2016;11:e0164066 pubmed publisher
    ..To investigate whether Opa1 (protein involved in mitochondrial inner-membrane fusion) deficiency affects I/R injuries...
  62. Ramírez S, Gómez Valadés A, Schneeberger M, Varela L, Haddad Tóvolli R, Altirriba J, et al. Mitochondrial Dynamics Mediated by Mitofusin 1 Is Required for POMC Neuron Glucose-Sensing and Insulin Release Control. Cell Metab. 2017;25:1390-1399.e6 pubmed publisher
    ..Collectively, our data posit MFN1-mediated mitochondrial dynamics in POMC neurons as an intrinsic nutrient-sensing mechanism and unveil an unrecognized link between this subset of neurons and insulin release. ..
  63. Augustin M, Sedlmeier R, Peters T, Huffstadt U, Kochmann E, Simon D, et al. Efficient and fast targeted production of murine models based on ENU mutagenesis. Mamm Genome. 2005;16:405-13 pubmed
    ..We conclude that ENU-based targeted mutagenesis is a powerful tool for the fast and high-throughput production of murine gene-specific models for biomedical research. ..
  64. Liu W, Tian F, Kurata T, Morimoto N, Abe K. Dynamic changes of mitochondrial fusion and fission proteins after transient cerebral ischemia in mice. J Neurosci Res. 2012;90:1183-9 pubmed publisher
    ..showed that mitochondrial fission protein dynamin-related protein 1 (Drp1) and fusion protein optic atrophy 1 (Opa1) were both upregulated in the ischemic penumbra, with the peak at 2 days after tMCAO, whereas phosphorylated-Drp1 (..
  65. Ramonet D, Perier C, Recasens A, Dehay B, Bove J, Costa V, et al. Optic atrophy 1 mediates mitochondria remodeling and dopaminergic neurodegeneration linked to complex I deficiency. Cell Death Differ. 2013;20:77-85 pubmed publisher
    ..Optic atrophy 1 (OPA1), a dynamin-related GTPase of the inner mitochondrial membrane, participates in mitochondrial fusion and apoptotic ..