Web22 de nov. de 2024 · This work investigated the effect of thyroxine on the biogenesis and quality control system in rat heart mitochondria. In hyperthyroid rats, the concentrations of free triiodothyronine and thyroxine increased severalfold, indicating the development of hyperthyroidism in these animals. The electron microscopy showed 58% of cardiac … Web9 de jul. de 2024 · The OPA1 gene is composed of 30 coding exons distributed across more than 90 kb of genomic DNA. Alternative splicing of exons 4, 4b and 5b gives rise to eight different isoforms with open reading frames for polypeptides of 960 to …
OPA1 deficiency promotes secretion of FGF21 from muscle that
Web15 de jan. de 2024 · OPA1, a large GTPase of the dynamin superfamily, mediates fusion of the mitochondrial inner membranes, regulates cristae morphology, and maintains respiratory chain function. Inner membrane-anchored long forms of OPA1 (l-OPA1) are proteolytically processed by the OMA1 or YME1L proteases, acting at c … Web10 de jan. de 2024 · Using an in vitro reconstitution system, we describe a mechanism for how mitochondrial inner-membrane fusion is regulated by the ratio of two forms of Opa1. We found that the long-form of Opa1 (l-Opa1) is sufficient for membrane docking, hemifusion and low levels of content release. However, stoichiometric levels of the processed, short … tsx performance graph
Mutation spectrum of the OPA1 gene in a large cohort of patients …
WebOPA1 is a dominantly inherited optic neuropathy occurring in 1 in 50,000 individuals that features progressive loss in visual acuity leading, in many cases, to legal blindness. Defects in OPA1 are the cause of optic atrophy 1 with deafness (OPA1D) [MIM:125250]. Some individuals with mutations in OPA1 manifest also ophthalmoplegia and myopathy. Web25 de set. de 2013 · Mitochondrial quality control is fundamental to all neurodegenerative diseases, including the most prominent ones, Alzheimer’s Disease and Parkinsonism. It is accomplished by mitochondrial network dynamics – continuous fission and fusion of mitochondria. Mitochondrial fission is facilitated by DRP1, while MFN1 and MFN2 on the … Web21 de mar. de 2024 · Molecular mass: 111631 Da Protein existence level: PE1 Quaternary structure: Oligomeric complex consisting of membrane-bound and soluble forms of … phoebe apartments pa