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Description
Function: Dynamin-related GTPase required for mitochondrial fusion and regulation of apoptosis. May form a diffusion barrier for proteins stored in mitochondrial cristae. Proteolytic processing in response to intrinsic apoptotic signals may lead to disassembly of OPA1 oligomers and release of the caspase activator cytochrome C (CYCS) into the mitochondrial intermembrane space. Defects in OPA1 are a cause of optic atrophy type 1 (OPA1). OPA1 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. Some individuals with mutations in OPA1 manifest also ophthalmoplegia and myopathy.Subcellular location: Mitochondrion inner membrane, Single-pass membrane protein. Mitochondrion intermembrane spaceTissue specificity: Highly expressed in retina. Also expressed in brain, testis, heart and skeletal muscle. Isoform 1 expressed in retina, skeletal muscle, heart, lung, ovary, colon, thyroid gland, leukocytes and fetal brain. Isoform 2 expressed in colon, liver, kidney, thyroid gland and leukocytes. Low levels of all isoforms expressed in a variety of tissues. Also known as: Dynamin-like 120 kDa protein, mitochondrial, Optic atrophy protein 1, Dynamin-like 120 kDa protein, form S1.
Immunogen
A synthetic peptide from n-terminal region of human OPA1 (Optic atrophy protein 1) conjugated to an immunogenic carrier protein was used as the antigen.