| protein
| requiring MFN1 to induce mitochondrial fusion (but not MFN2) |
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directly interacts with subunits of complexes I, II and III, but not IV and with apoptosis inducing factor  |
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a subset of OPA1 isoforms is constitutively cleaved by YME1L1 |
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interacts with BNIP3, leading to mitochondrial fragmentation and apoptosis |
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organizes a supramolecular complex containing both PKA and perilipin ( |
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can associate with dynamin-like GTPase Optic Atrophy-1 (OPA1) and contribute to the maintenance of mitochondrial morphology |
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Higd-1a |
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stress-induced OPA1 processing by OMA1 converts OPA1 completely into short isoforms, inhibits fusion, and triggers mitochondrial fragmentation |
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OMA1 is a critical regulator of neuronal survival, demonstrating that stress-induced OPA1 processing by OMA1 promotes neuronal death and neuroinflammatory responses |
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differential degradation of YME1L1 and OMA1 alters their proteolytic processing of the dynamin-like GTPase OPA1, a critical regulator of mitochondrial inner membrane morphology, which influences the recovery of tubular mitochondria following membrane-depolarization-induced fragmentation |
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SLC25A46 interacts with MFN2, OPA1, and the mitochondrial contact site and cristae organizing system (MICOS) complex |
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SIRT4 interacts physically with OPA1, and SIRT4-OPA1 axis is causally linked to mitochondrial dysfunction and altered mitochondrial dynamics that translates into aging-associated decreased mitophagy based on an unbalanced mitochondrial fusion/fission cycle |
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| mouse homozygous for a Opa1 gene splice site mutation (c.1065 + 5G>A) die in utero during embryogenesis with first notable developmental delay at E8.5 while heterozygous mice are viable but exhibit an age-dependent loss of retinal ganglion cells progressesing to a severe degeneration of the ganglion cell and nerve fibre layer whose axones display an abnormal shape, disorganized mitochondrial cristae structures | |
ENU-induced mutant mouse carrying a protein-truncating nonsense mutation in opa1 display 50% reduction in opa1 protein in retina and all tissues on western analysis, alteration in morphology, with an increase in mitochondrial fission and fragmentation, a slow onset of degeneration in the optic nerve and a functional reduction in visual function |
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Mouse Opa1 homozygous mutation is embryonic lethal by 13.5 days post coitum |
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Heterozygous mutation of dOpa1 by a P-element or transposon insertions causes no discernable eye phenotype in Drosophila while homozygous mutation results in embryonic lethality |
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somatic homozygous mutation of dOpa1 in the Drosophila eyes caused rough (mispatterning) due to the loss of hexagonal lattice cells in developing eyes, and glossy (decreased lens and pigment deposition) eye phenotypes in adult flies |
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in adult flies, the dOpa1 mutation caused an increase in reactive oxygen species production as well as mitochondrial fragmentation associated with loss and damage of the cone and pigment cells |
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heterozygous dOpa1 mutation caused shortened lifespan, increased susceptibility to oxidative stress and elevated production of ROS in the whole Drosophila |
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homozygous mutant drosophila for OPA1 developed a rough and glossy eye phenotype due to the loss of hexagonal lattice cells, with decreased lens and pigment deposition |
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heterozygous B6;C3-Opa1(Q285STOP) mice display retinal ganglion cell dendropathy |
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Opa1(+/-) mutant mice (B6;C3-Opa1) have a marked reduction in retinal ganglion cell synaptic, decreased levels of postsynaptic density protein 95 and glutamatergic synaptic sites, increased synaptic vesicle number in bipolar cell terminal, retraction of mitochondria towards the soma of retinal ganglion cells and mitochondrial fragmentation, preceding dendritic loss |
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OPA1 knockdown retinal ganglion cells results in profound cristae depletion and loss of crista junctions, and defective Ca(2+) homeostasis |
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Opa1 mouse model carrying the recurrent Opa1(delTTAG) mutation displays a multi-systemic poly-degenerative phenotype: visual failure, deafness, encephalomyopathy, peripheral neuropathy, ataxia and cardiomyopathy, premature age-related axonal and myelin degenerations, increased autophagy and mitophagy and mitochondrial supercomplex instability preceding degeneration and cell death |
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downregulation of the OPA1 protein in rodent cortical primary neurons leads to fragmented mitochondria that become less abundant along the dendrites, reduced expression of mitochondrial respiratory complexe, reduction of mitochondrial DNA, decreased mitochondrial membrane potential, diminished reactive oxygen species levels, and a major restriction of dendritic growth, together with reduction of synaptic proteins |
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depletion of Opa1 in zebrafish embryos leads to mitochondrial morphology disruption, abnormal blood circulation and heart defects, small eyes, small pectoral fin buds and impairment of locomotor activity |
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Ablation of OPA1 in HeLa cells and 143b cells specifically abrogated pH flashes and reduced the propagation of matrix photoactivated GFP |
