{"product_id":"opa1-rabbit-polyclonal-antibody-es11922","title":"OPA1 Rabbit Polyclonal Antibody - ES11922","description":"\u003cp\u003eOPA1 Rabbit Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: ES11922-50, ES11922-100\u003c\/p\u003e\n\n\u003cp\u003eLead times: approximately 7-10 business days, if manufacturer has product in stock\u003c\/p\u003e\n\n\u003cp\u003eManufacturer\/Ship Location: China\u003c\/p\u003e\n\n\u003cp\u003eBackground: 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. [provided by RefSeq, Mar 2009],\u003c\/p\u003e \n\n\u003cp\u003eReactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB, ELISA\u003c\/p\u003e\n\n\u003cp\u003eClonality: Polyclonal\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Synthesized peptide derived from part region of human protein\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003eTarget: OPA1,\u003c\/p\u003e \n\n\u003cp\u003eSpecificity: OPA1 Polyclonal Antibody detects endogenous levels of protein.\u003c\/p\u003e\n\n\u003cp\u003eDilution: WB 1:500-2000 ELISA 1:5000-20000\u003c\/p\u003e\n\n\u003cp\u003ePurification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.\u003c\/p\u003e\n\n\u003cp\u003eConcentration: 1 mg\/ml,\u003c\/p\u003e \n\n\u003cp\u003eFormulation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.\u003c\/p\u003e\n\n\u003cp\u003eExpression: 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.\u003c\/p\u003e\n\n\u003cp\u003eObserved Band (KD): 105kD\u003c\/p\u003e\n\n\u003cp\u003eFields: Spinocerebellar ataxia\u003c\/p\u003e\n\n\u003cp\u003eFunction: Disease: Defects in OPA1 are a cause of optic atrophy type 1 (OPA1) [MIM:165500]. 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. Disease: Defects in OPA1 are the cause of optic atrophy 1 and deafness [MIM:125250]. Some individuals with mutations in OPA1 manifest also ophthalmoplegia and myopathy. 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. PTM: PARL-dependent proteolytic processing releases an antiapoptotic soluble form not required f\u003c\/p\u003e\n\n\u003cp\u003eSubcellular Location: Mitochondrion inner membrane, Single-pass membrane protein Mitochondrion intermembrane space Mitochondrion membrane Detected at contact sites between endoplasmic reticulum and mitochondrion membranes.\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: -20°C\/1 year\u003c\/p\u003e\n\n\u003cp\u003eGene Name: OPA1, KIAA0567\u003c\/p\u003e\n\n\u003cp\u003eProtein Name: Dynamin-like 120 kDa protein, mitochondrial, Optic atrophy protein 1 [Cleaved into: Dynamin-like 120 kDa protein, form S1]\u003c\/p\u003e\n\n\u003cp\u003eHuman Gene ID: 4976\u003c\/p\u003e\n\n\u003cp\u003eHuman SwissProt Number: O60313\u003c\/p\u003e\n\n\u003cp\u003eMouse SwissProt Number: P58281\u003c\/p\u003e\n\n\u003cp\u003eRat SwissProt Number: Q2TA68\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":45825500086451,"sku":"ES11922-50","price":186.0,"currency_code":"CAD","in_stock":true},{"title":"100μL","offer_id":45825500119219,"sku":"ES11922-100","price":372.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/elk-biotechnology-logo-product.jpg?v=1786476023","url":"https:\/\/afsbio.com\/products\/opa1-rabbit-polyclonal-antibody-es11922","provider":"AFSBio Inc.","version":"1.0","type":"link"}