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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 (zeige TNFRSF1B Proteine) 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 (zeige INS Proteine) resistance, by mechanisms that involve activation of endoplasmic reticulum stress and secretion of fibroblast growth factor 21 (FGF21 (zeige FGF21 Proteine)) from skeletal muscle, resulting in increased metabolic rates and improved whole-body insulin (zeige INS Proteine) 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 (zeige CA2 Proteine)+ uptake, and subsequent increase in NCX (zeige SLC8A1 Proteine) activity.
Whereas Parkin (zeige PARK2 Proteine) has been reported to positively regulate the expression of OPA1 through NEMO (zeige IKBKG Proteine), herein we found that PARK2 (zeige PARK2 Proteine) overexpression did not modify the expression of OPA1.
stress-induced OMA1 (zeige OMA1 Proteine) 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 (zeige YME1L1 Proteine), are altered; in vitro and in vivo, OPA1 is cleaved to shorter forms and Yme1L (zeige YME1L1 Proteine) expression is reduced.
results indicate that the OPA1-dependent cristae remodeling pathway is a fundamental, targetable determinant of tissue damage in vivo.
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 (zeige RACGAP1 Proteine) 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.
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