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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 (show TNFRSF1B Proteins) 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 (show INS Proteins) resistance, by mechanisms that involve activation of endoplasmic reticulum stress and secretion of fibroblast growth factor 21 (FGF21 (show FGF21 Proteins)) from skeletal muscle, resulting in increased metabolic rates and improved whole-body insulin (show INS Proteins) 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 (show CA2 Proteins)+ uptake, and subsequent increase in NCX (show SLC8A1 Proteins) activity.
Whereas Parkin (show PARK2 Proteins) has been reported to positively regulate the expression of OPA1 through NEMO (show IKBKG Proteins), herein we found that PARK2 (show PARK2 Proteins) overexpression did not modify the expression of OPA1.
stress-induced OMA1 (show OMA1 Proteins) activation and guanosine triphosphatase OPA1 cleavage limit mitochondrial fusion and promote neuronal death
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 (show DNM1 Proteins)-related GTPase (show RACGAP1 Proteins) 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 (show Plxna2 Proteins) 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 (show Plxna2 Proteins) 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 (show SIRT4 Proteins)-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 (show MFN1 Proteins) 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.
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