| 产品详情 |
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| Antigenic Specificity | Optic Atrophy 1 (Autosomal Dominant) (OPA1) (N-Term) |
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| Clone | polyclonal |
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| Host Species | Rabbit |
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| Reactive Species | human, rat, mouse |
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| Isotype | n/a |
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| Format | serum |
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| Size | 100 µL |
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| Concentration | n/a |
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| Applications | Immunohistochemistry (IHC),Western Blotting (WB) |
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| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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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. |
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| 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. |
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| Other Names | 1200011N24Rik|AI225888|AI847218|lilr3|mKIAA0567|MGM1|NPG|NTG|largeG|fk62d06|wu:fb77a10|wu:fk62d06|zgc:92092 |
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| Gene, Accession # | Gene ID: 4976,74143,171116 |
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| Catalog # | ABIN571614 |
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| Price | |
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| Order / More Info | Optic Atrophy 1 (Autosomal Dominant) (OPA1) (N-Term) Antibody from ANTIBODIES-ONLINE GmbH |
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| Product Specific References | n/a |
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