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Propose a novel function of OPA1 in mediating the CoQ10-responsive regulation of respiratory CIV activity in brain mitochondria from aging mice.
multiple OPA1 isoforms are required for mitochondrial dynamics, while any single isoform can support all other functions.
Mechanistically, the ablation of Opa1 leads to ER stress, which signals via the unfolded protein response (UPR) and FoxOs, inducing a catabolic program of muscle loss and systemic aging.
Mitochondrial dysfunction in an Opa1 mouse model of dominant optic atrophy results from Opa1 haploinsufficiency.
TNFR2 activation protects cardiac myocytes against stress by up-regulating OPA1 expression.
OPA1 mutant mice are resistant to age- and diet-induced weight gain and insulin resistance, by mechanisms that involve activation of endoplasmic reticulum stress and secretion of fibroblast growth factor 21 (FGF21) from skeletal muscle, resulting in increased metabolic rates and improved whole-body insulin sensitivity.
OPA1 modulates cristae morphology but is dispensable for cristae junction formation. Endogenous OPA1 and MIC60 show a physical interaction.
Opa1 deficiency was associated with increased sensitivity to Ischemia-Reperfusion Injuries, imbalance in dynamic mitochondrial Ca2+ uptake, and subsequent increase in NCX activity.
Whereas Parkin has been reported to positively regulate the expression of OPA1 through NEMO, herein we found that PARK2 overexpression did not modify the expression of OPA1.
stress-induced OMA1 activation and guanosine triphosphatase OPA1 cleavage limit mitochondrial fusion and promote neuronal death
Data suggest that in a mouse model of neonatal hypoxic-ischemic brain injury, the expression of mitochondrial shaping proteins, such as OPA1 and Yme1L, are altered; in vitro and in vivo, OPA1 is cleaved to shorter forms and Yme1L expression is reduced.
results indicate that the OPA1-dependent cristae remodeling pathway is a fundamental, targetable determinant of tissue damage in vivo.
cristae shape amelioration by controlled Opa1 overexpression improves two mouse models of mitochondrial disease.
unprocessed OPA1 is sufficient to maintain heart function, OMA1 is a critical regulator of cardiomyocyte survival, and mitochondrial morphology and cardiac metabolism are intimately linked.
Data suggest that mitochondrial fusion and fission events are regulated by four GTPases: OPA1, Drp1 (dynamin 1-like protein), and Mfn1/2 (mitofusin 1 and 2). [REVIEW]
Photoresponsive RGCs are protected against cell death due to the Opa1 mutation, but not by melanopsin expression itself.
Results suggest that Mfn2 and OPA1 are upregulated during bone marrow progenitor differentiation and promote the migration of immature dendritic cells by regulating the expression of CCR7.
Reactive oxygen species might affect MyHC content by modulating OPA1 cleavage in muscle degeneration.
mitochondria adapt to metabolic shifts through the parallel remodeling of the cristae and of the MERCs via a mechanism that degrades Opa1 in an Mfn2-dependent pathway.
a novel way in which OPA1 senses energy substrate availability, which modulates its function in the regulation of mitochondrial architecture in a SLC25A protein-dependent manner.
Our data indicate that the LEU396ARG mutation in OPA1 is associated with severe dominant optic atrophy.
OPA1 gene therapy prevents retinal ganglion cell loss in a dominant optic atrophy mouse model.
We have generated a human iPSC line IISHDOi003-A from fibroblasts of a patient with a dominant optic atrophy 'plus' phenotype, harbouring a heterozygous mutation, c.1635C>A; p.Ser545Arg, in the OPA1 gene.
OPA1 is a dynamin-related GTPase that controls mitochondrial dynamics, cristae integrity, energetics and mitochondrial DNA maintenance, with eight isoforms being characterized. (Review)
Reports an assessment of the afferent visual system and OCT examination in an Italian cohort of fifty-two fully penetrant probands affected by Autosomal Dominant Optic Atrophy (ADOA) with OPA1 mutations and eight asymptomatic carriers of OPA1 mutations. Visual acuity and OCT data in missense mutations were compared with those associated with mutations inducing haploinsufficiency, and correlated with age in both groups.
we propose that the 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.
Data indicate genotype-phenotype correlations between various types of optic Atrophy 1 (OPA1) mutation and mitophagy.
The results show that a metabolic shift from glycolysis in young to mitochondrial respiration in old normal human fibroblasts occurs during chronological lifespan, and MFN1 and OPA1 regulate this process.
Genetic testing identified disease-causing mutations in 34% of referred cases, with the majority of these in OPA1. Patients with mutations in OPA1 were more likely to have a family history of disease; however, 30.4% of patients without a family history were also found to have an OPA1 mutation.
OPA1 gene mutations were identified in Han Chinese patients with suspected Optic Neuropathy.
Identification of genomic rearrangements or pathogenic variants of OPA1 is meaningful for disease prognosis and proper genetic counseling in DOA consultation.
In brown adipocytes indirect evidence support the notion that OPA1 regulation of fission serves to increase thermogenesis, which thereby contributes to dissipation of energy.
Stabilization of OPA1 impedes cristae remodeling.
Our combination of proteomics, biochemistry, genetics, and electron tomography provides a unifying model for mammalian cristae biogenesis by OPA1 and MICOS.
The splice site mutation (c.985G>T) identified in the present study led to exon 10 skipping (c.985_1065del, p.V329_D355del), suggesting loss-of-function of the GTPase domain of the OPA1 protein, which is likely to cause haplo-insufficiency, a major disease mechanism in DOA.
study identifies a novel pathogenic OPA1 mutation and shows that it is located in the transcript region not prone for NMD activation
OPA1 gene screening in patients with bilateral optic atrophy is an important part of clinical evaluation as it may establish correct clinical diagnosis
OPA1 and cardiolipin cooperate in heterotypic mitochondrial inner membrane fusion.
We propose that OPA1 stabilizes respiratory chain supercomplexes in a conformation that enables respiring mitochondria to compensate a drop in Deltapsim by an explosive matrix pH flash.
We report the first cases of genetically confirmed OPA1-related autosomal-dominant optic atrophy from Singapore, including a novel mutation causing 'ADOA plus' syndrome.
Opa1 is required for proper mitochondrial metabolism in early development
This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Mutations in this gene have been associated with optic atrophy type 1, which is a dominantly inherited optic neuropathy resulting in progressive loss of visual acuity, leading in many cases to legal blindness. Multiple transcript variants encoding different isoforms have been found for this gene.
dynamin-like 120 kDa protein, mitochondrial
, large GTP-binding protein
, optic atrophy 1 homolog
, optic atrophy protein 1 homolog
, RN protein
, optic atrophy 1 (autosomal dominant)
, optic atrophy 1-like protein
, dynamin-like guanosine triphosphatase
, mitochondrial dynamin-like GTPase
, optic atrophy protein 1