产品详情
简单介绍：
Anti-ERAB (E135) Antibody
详情介绍：
Overview
| Name: | Anti-ERAB (E135) Antibody See all ERAB primary antibodies
|
| Description: | Rabbit polyclonal antibody to ERAB (E135) |
| Specificity: | ERAB (E135) pAb detects endogenous levels of ERAB protein. |
| Applications: | WB, IHC |
| Reactivity: | Human, Mouse, Rat |
| Immunogen: | Synthetic peptide, corresponding to amino acids 102-150 of Human ERAB. |
| Host: | Rabbit |
| Clonality: | Polyclonal |
| Conjugate: | Unconjugated |
| Molecular Weight: | ~ 27 kDa |
| Purity: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE). |
| Product Form: | 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2. |
Target
| Function: | Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. Catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone. Catalyzes the third step in the beta-oxidation of fatty acids. Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. Also exhibits 20-beta-OH and 21-OH dehydrogenase activities with C21 steroids. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). |
| Tissue Specificity: | Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD. |
| Involvement in Disease: | 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: A disorder that leads to neurological abnormalities, including psychomotor retardation and, in virtually all patients, loss of mental and motor skills. Mental retardation, X-linked, syndromic, 10: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. MRXS10 patients manifest mild mental retardation, choreoathetosis and abnormal behavior. Mental retardation, X-linked 17: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. Intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation, while syndromic mental retardation presents with associated physical, neurological and/or psychiatric manifestations. |
| Sequence Similarities: | Belongs to the short-chain dehydrogenases/reductases (SDR) family. |
| Cellular Location: | Mitochondrion. |
| Database Links: |
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| Synonyms: | |
| Information: | Target information shown above is from the UniProt Consortium. |
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