Anti-Zebrafish Opa1 / Dynamin-like GTPase Opa1

0.5mg/ml if reconstituted with 0.2ml sterile DI water. Dynamin-like 120 kDa protein, mitochondrial is a protein that in humans is encoded by the OPA1 gene. It is mapped to 3q29. This protein regulates mitochondrial fusion and cristae structure in the inner mitochondrial membrane (IMM) and contributes to ATP synthesis and apoptosis. 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. Protein function: Dynamin-related GTPase that is essential for normal mitochondrial morphology by mediating fusion of the mitochondrial inner membranes, regulating cristae morphology and maintaining respiratory chain function. Exists in two forms: the transmembrane, long form (Dynamin-like GTPase OPA1, long form, L-OPA1), which is tethered to the inner mitochondrial membrane, and the short soluble form (Dynamin-like GTPase OPA1, short form, S-OPA1), which results from proteolytic cleavage and localizes in the intermembrane space. Both forms (L-OPA1 and S-OPA1) cooperate to catalyze the fusion of the mitochondrial inner membrane. The equilibrium between L-OPA1 and S-OPA1 is essential: excess levels of S-OPA1, produced by cleavage by OMA1 following loss of mitochondrial membrane potential, lead to an impaired equilibrium between L-OPA1 and S-OPA1, inhibiting mitochondrial fusion. The balance between L-OPA1 and S-OPA1 also influences cristae shape and morphology. Its role in mitochondrial morphology is required for mitochondrial genome maintenance. [The UniProt Consortium]