Gene Symbol: Opa1
Description: Optic atrophy 1
Alias: CG8479, Dmel\CG8479, OPA1, OPA1-like, Opa1-like, dOPA1, dOpa1, l(2)s3475, opa1, opa1-like, optic atrophy 1, CG8479-PA, CG8479-PB, CG8479-PC, CG8479-PD, CG8479-PE, Opa1-PA, Opa1-PB, Opa1-PC, Opa1-PD, Opa1-PE, Opa1-like, dOpa1, drosophila optic atrophy 1-like, lethal (2) s3475, opa1, optic atrophy 1 ortholog, optic atrophy 1-like
Species: fruit fly

Top Publications

  1. Dorn G, Clark C, Eschenbacher W, Kang M, Engelhard J, Warner S, et al. MARF and Opa1 control mitochondrial and cardiac function in Drosophila. Circ Res. 2011;108:12-7 pubmed publisher
    ..Live imaging analysis revealed that heart tube-specific knockdown of MARF or Opa1 increases mitochondrial morphometric heterogeneity and induces heart tube dilation with profound contractile ..
  2. Poole A, Thomas R, Andrews L, McBride H, Whitworth A, Pallanck L. The PINK1/Parkin pathway regulates mitochondrial morphology. Proc Natl Acad Sci U S A. 2008;105:1638-43 pubmed publisher
    ..gene dosage and by heterozygous loss-of-function mutations affecting the mitochondrial fusion-promoting factors OPA1 and Mfn2...
  3. Yang Y, Ouyang Y, Yang L, Beal M, McQuibban A, Vogel H, et al. Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery. Proc Natl Acad Sci U S A. 2008;105:7070-5 pubmed publisher
    ..core components of the mitochondrial fission/fusion machinery, including the dynamin-like GTPases Drp1, Mitofusin, Opa1, and the Drp1-interacting protein Fis1, have been identified...
  4. Poole A, Thomas R, Yu S, Vincow E, Pallanck L. The mitochondrial fusion-promoting factor mitofusin is a substrate of the PINK1/parkin pathway. PLoS ONE. 2010;5:e10054 pubmed publisher
    ..abundance of the mitochondrial fission-promoting factor Drp1 or the mitochondrial fusion-promoting factor Opa1, or the subcellular distribution of Drp1...
  5. Park J, Lee G, Chung J. The PINK1-Parkin pathway is involved in the regulation of mitochondrial remodeling process. Biochem Biophys Res Commun. 2009;378:518-23 pubmed publisher
    ..with the regulators of mitochondrial fusion and fission such as Drp1, which promotes mitochondrial fission, and Opa1 or Marf, which induces mitochondrial fusion...
  6. Lutz A, Exner N, Fett M, Schlehe J, Kloos K, Lämmermann K, et al. Loss of parkin or PINK1 function increases Drp1-dependent mitochondrial fragmentation. J Biol Chem. 2009;284:22938-51 pubmed publisher
    ..caused by either parkin or PINK1 loss of function could be rescued by the mitochondrial fusion proteins Mfn2 and OPA1 or by a dominant negative mutant of the fission protein Drp1...
  7. Ziviani E, Tao R, Whitworth A. Drosophila parkin requires PINK1 for mitochondrial translocation and ubiquitinates mitofusin. Proc Natl Acad Sci U S A. 2010;107:5018-23 pubmed publisher
    ..We hypothesize that Mfn ubiquitination may provide a mechanism by which terminally damaged mitochondria are labeled and sequestered for degradation by autophagy. ..
  8. Park J, Kim Y, Choi S, Koh H, Lee S, Kim J, et al. Drosophila Porin/VDAC affects mitochondrial morphology. PLoS ONE. 2010;5:e13151 pubmed publisher
    ..Collectively, our study showed that loss of Drosophila Porin results in mitochondrial morphological defects and suggested that the defective mitochondrial function by Porin deficiency affects the mitochondrial remodeling process. ..
  9. Deng H, Dodson M, Huang H, Guo M. The Parkinson's disease genes pink1 and parkin promote mitochondrial fission and/or inhibit fusion in Drosophila. Proc Natl Acad Sci U S A. 2008;105:14503-8 pubmed publisher
    ..by a dynamic balance between the opposing actions of mitochondrial fusion, controlled by Mitofusin (mfn) and Optic atrophy 1 (opa1), and mitochondrial fission, controlled by drp1...

More Information


  1. Tang S, Le P, Tse S, Wallace D, Huang T. Heterozygous mutation of Opa1 in Drosophila shortens lifespan mediated through increased reactive oxygen species production. PLoS ONE. 2009;4:e4492 pubmed publisher
    b>Optic atrophy 1 (OPA1) is a dynamin-like GTPase located in the inner mitochondrial membrane and mutations in OPA1 are associated with autosomal dominant optic atrophy (DOA)...
  2. McQuibban G, Lee J, Zheng L, Juusola M, Freeman M. Normal mitochondrial dynamics requires rhomboid-7 and affects Drosophila lifespan and neuronal function. Curr Biol. 2006;16:982-9 pubmed
    ..We also find that mutations in Drosophila optic atrophy 1-like (Opa1-like), the ortholog of yeast mgm1, display similar phenotypes, suggesting a shared role for ..
  3. Yarosh W, Monserrate J, Tong J, Tse S, Le P, Nguyen K, et al. The molecular mechanisms of OPA1-mediated optic atrophy in Drosophila model and prospects for antioxidant treatment. PLoS Genet. 2008;4:e6 pubmed publisher
    Mutations in optic atrophy 1 (OPA1), a nuclear gene encoding a mitochondrial protein, is the most common cause for autosomal dominant optic atrophy (DOA)...
  4. Sandoval H, Yao C, Chen K, Jaiswal M, Donti T, Lin Y, et al. Mitochondrial fusion but not fission regulates larval growth and synaptic development through steroid hormone production. elife. 2014;3: pubmed publisher
    ..Our data show that Marf and Mitofusins share an evolutionarily conserved role in mitochondrial transport, cholesterol ester storage and steroid-hormone synthesis. ..
  5. Vilain S, Esposito G, Haddad D, Schaap O, Dobreva M, Vos M, et al. The yeast complex I equivalent NADH dehydrogenase rescues pink1 mutants. PLoS Genet. 2012;8:e1002456 pubmed publisher
  6. Boyle M, French R, Cosand K, Dorman J, Kiehart D, Berg C. Division of labor: subsets of dorsal-appendage-forming cells control the shape of the entire tube. Dev Biol. 2010;346:68-79 pubmed publisher
    ..Gbeta mutant floor cells are unable to control the width of the appendage while Gbeta mutant leading roof cells fail to direct the elongation of the appendage and the convergent-extension of the roof-cell population. ..
  7. Huang Y, Xie J, Wang T. A Fluorescence-Based Genetic Screen to Study Retinal Degeneration in Drosophila. PLoS ONE. 2015;10:e0144925 pubmed publisher
    ..Our findings demonstrate that this "Rh1::GFP ey-flp/hid" method enables high-throughput F1 genetic screens to rapidly and precisely identify mutations of retinal degeneration. ..
  8. Rival T, Macchi M, Arnauné Pelloquin L, Poidevin M, Maillet F, Richard F, et al. Inner-membrane proteins PMI/TMEM11 regulate mitochondrial morphogenesis independently of the DRP1/MFN fission/fusion pathways. EMBO Rep. 2011;12:223-30 pubmed publisher
    ..Mitofusins (MFNs) and OPA1 proteins are dynamin-related GTPases that are required for outer- and inner-mitochondrial membrane fusion ..
  9. Huang Z, Ren S, Jiang Y, Wang T. PINK1 and Parkin cooperatively protect neurons against constitutively active TRP channel-induced retinal degeneration in Drosophila. Cell Death Dis. 2016;7:e2179 pubmed publisher
    ..In addition, the results demonstrated that maintaining mitochondrial homeostasis via PINK1/Parkin-dependent mitochondrial quality control can potentially alleviate cell death in a wide range of neurodegenerative diseases. ..
  10. Tanner E, Blute T, Brachmann C, McCall K. Bcl-2 proteins and autophagy regulate mitochondrial dynamics during programmed cell death in the Drosophila ovary. Development. 2011;138:327-38 pubmed publisher
    ..Furthermore, Bcl-2 family mutants show a striking defect in cell death in the ovary. These data indicate that a mitochondrial pathway is a major mechanism for activation of cell death in Drosophila oogenesis. ..
  11. Debattisti V, Pendin D, Ziviani E, Daga A, Scorrano L. Reduction of endoplasmic reticulum stress attenuates the defects caused by Drosophila mitofusin depletion. J Cell Biol. 2014;204:303-12 pubmed publisher
    ..and ER morphology and triggering ER stress that was conversely absent in flies lacking the fusion protein optic atrophy 1. Expression of Mfn2 and ER stress reduction in flies lacking Marf corrected ER shape, attenuating the ..
  12. Wiemerslage L, Lee D. Quantification of mitochondrial morphology in neurites of dopaminergic neurons using multiple parameters. J Neurosci Methods. 2016;262:56-65 pubmed publisher
    ..We suggest at least four variables should be considered when using mitochondrial structure as an experimental endpoint. ..
  13. Yu Y, Lee H, Chen K, Suhan J, Qiu M, Ba Q, et al. Inner membrane fusion mediates spatial distribution of axonal mitochondria. Sci Rep. 2016;6:18981 pubmed publisher
    ..Disruption of inner membrane fusion by knockdown of dOpa1, Drosophila Optic Atrophy 1, not only increased the spatial density of stationary and moving mitochondria but also changed their spatial ..
  14. Macchi M, El Fissi N, Tufi R, Bentobji M, Liévens J, Martins L, et al. The Drosophila inner-membrane protein PMI controls crista biogenesis and mitochondrial diameter. J Cell Sci. 2013;126:814-24 pubmed publisher
    ..Since PMI and its human orthologue TMEM11 are regulators of mitochondrial morphology, our data suggest that, by controlling crista length, PMI influences mitochondrial diameter and tubular shape. ..
  15. Freeman M. The rhomboid-like superfamily: molecular mechanisms and biological roles. Annu Rev Cell Dev Biol. 2014;30:235-54 pubmed publisher
    ..Their potential medical significance is now becoming apparent in several areas. ..
  16. Banerjee S, Chinthapalli B. A proteomic screen with Drosophila Opa1-like identifies Hsc70-5/Mortalin as a regulator of mitochondrial morphology and cellular homeostasis. Int J Biochem Cell Biol. 2014;54:36-48 pubmed publisher
    ..The mammalian Optic atrophy 1 (OPA1) that functions in mitochondrial fusion is associated with Optic Atrophy and has been implicated in ..
  17. Rahman M, Kylsten P. Rhomboid-7 over-expression results in Opa1-like processing and malfunctioning mitochondria. Biochem Biophys Res Commun. 2011;414:315-20 pubmed publisher
    ..In the CNS, elevated levels of rho-7 were accompanied by both isoforms of Opa1-like, a dynamin-like GTPase, a mitochondrial component involved in regulating mitochondrial dynamics and function, ..
  18. Shahrestani P, Leung H, Le P, Pak W, Tse S, Ocorr K, et al. Heterozygous mutation of Drosophila Opa1 causes the development of multiple organ abnormalities in an age-dependent and organ-specific manner. PLoS ONE. 2009;4:e6867 pubmed publisher
    b>Optic Atrophy 1 (OPA1) is a ubiquitously expressed dynamin-like GTPase in the inner mitochondrial membrane. It plays important roles in mitochondrial fusion, apoptosis, reactive oxygen species (ROS) and ATP production...
  19. Deng Q, Guo T, Zhou X, Xi Y, Yang X, Ge W. Cross-Talk Between Mitochondrial Fusion and the Hippo Pathway in Controlling Cell Proliferation During Drosophila Development. Genetics. 2016;203:1777-88 pubmed publisher
    ..In addition, depletion of other mitochondrial fusion components, Opa1 or Marf, inactivates the Hippo pathway as well...
  20. Fernandes C, Rao Y. Genome-wide screen for modifiers of Parkinson's disease genes in Drosophila. Mol Brain. 2011;4:17 pubmed publisher
    ..Among them, opa1 and drp1 have been previously implicated in the PD pathways, whereas debra (dbr), Pi3K21B and ?4GalNAcTA are novel ..
  21. Hwang R, Wiemerslage L, LaBreck C, Khan M, Kannan K, Wang X, et al. The neuroprotective effect of human uncoupling protein 2 (hUCP2) requires cAMP-dependent protein kinase in a toxin model of Parkinson's disease. Neurobiol Dis. 2014;69:180-91 pubmed publisher
  22. DUBOFF B, Götz J, Feany M. Tau promotes neurodegeneration via DRP1 mislocalization in vivo. Neuron. 2012;75:618-32 pubmed publisher
    ..Our results thus identify actin-mediated disruption of mitochondrial dynamics as a direct mechanism of tau toxicity in neurons in vivo...
  23. Urban S. Rhomboid proteins: conserved membrane proteases with divergent biological functions. Genes Dev. 2006;20:3054-68 pubmed
    ..Further study of these novel enzymes promises to reveal the evolutionary path of rhomboid protein function, which could provide insights into the forces that drive the molecular evolution of regulatory mechanisms. ..
  24. Rai M, Katti P, Nongthomba U. Drosophila Erect wing (Ewg) controls mitochondrial fusion during muscle growth and maintenance by regulation of the Opa1-like gene. J Cell Sci. 2014;127:191-203 pubmed publisher
    ..in Drosophila indirect flight muscles (IFMs) that the nuclear-encoded mitochondrial inner membrane fusion gene, Opa1-like, is regulated in a spatiotemporal fashion by the transcription factor/co-activator Erect wing (Ewg)...
  25. Rai M, Nongthomba U. Effect of myonuclear number and mitochondrial fusion on Drosophila indirect flight muscle organization and size. Exp Cell Res. 2013;319:2566-77 pubmed publisher
    ..Our results establish the organization levels of DLMs and highlight the importance of the appropriate number of nuclei and mitochondrial fusion in determining the overall organization, growth and size of DLMs. ..
  26. Nagaraj R, Gururaja Rao S, Jones K, Slattery M, Negre N, Braas D, et al. Control of mitochondrial structure and function by the Yorkie/YAP oncogenic pathway. Genes Dev. 2012;26:2027-37 pubmed publisher
    ..of Yorkie causes direct transcriptional up-regulation of genes that regulate mitochondrial fusion, such as opa1-like (opa1) and mitochondria assembly regulatory factor (Marf), and results in fused mitochondria with dramatic ..
  27. Tsubouchi A, Tsuyama T, Fujioka M, Kohda H, Okamoto Furuta K, Aigaki T, et al. Mitochondrial protein Preli-like is required for development of dendritic arbors and prevents their regression in the Drosophila sensory nervous system. Development. 2009;136:3757-66 pubmed publisher
    ..Our observations suggest that Prel-dependent regulation of mitochondrial activity is important for both growth and prevention of breakage of dendritic branches. ..
  28. Bhandari P, Song M, Dorn G. Dissociation of mitochondrial from sarcoplasmic reticular stress in Drosophila cardiomyopathy induced by molecularly distinct mitochondrial fusion defects. J Mol Cell Cardiol. 2015;80:71-80 pubmed publisher
    ..we previously observed that cardiomyocyte-specific genetic suppression of mitochondrial fusion factors optic atrophy 1 (Opa1) and mitofusin/MARF evokes cardiomyopathy in Drosophila hearts...