U-Blot® CYP27A1 Rabbit mAb
Ucallm_WR5411-50
Price range: $268.00 through $328.00
Product details
Background:cytochrome P450 family 27 subfamily A member 1(CYP27A1) Homo sapiens This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This mitochondrial protein oxidizes cholesterol intermediates as part of the bile synthesis pathway. Since the conversion of cholesterol to bile acids is the major route for removing cholesterol from the body, this protein is important for overall cholesterol homeostasis. Mutations in this gene cause cerebrotendinous xanthomatosis, a rare autosomal recessive lipid storage disease. [provided by RefSeq, Jul 2008],
Specifications
| Size | 50μL/100μL |
| Target | CYP27A1 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | 60kD |
| MW(Observed) | 55kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1660R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | CYP27A1 |
| Protein Name | Sterol 26-hydroxylase mitochondrial |
| Other Name | CYP27A1; / CYP27; / Sterol 26-hydroxylase; / mitochondrial; / 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 27-hydroxylase; / Cytochrome P-450C27/25; / Cytochrome P450 27; / Sterol 27-hydroxylase; / Vitamin D; / 3; / 25-hydroxylase |
| Species | Human |
| Gene ID | 1593 |
| Uniprot | Q02318, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q9DBG1 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Mitochondrion inner membrane; Peripheral membrane protein . Post-translationally targeted to mitochondria. All three of the receptor proteins in the TOM complex, TOMM70, TOMM20 and TOMM22 are required for the translocation across the mitochondrial outer membrane. After translocation into the matrix, associates with the inner membrane as a membrane extrinsic protein. . |
| Tissue Expression | Expressed in the neural retina and underlying retinal pigment epithelium (at protein level) (PubMed:21411718). Expressed in the gray and white matter of cerebellum (at protein level) (PubMed:28190002). |
| Signaling_pathway | Organismal Systems >> Endocrine system >> PPAR signaling pathway |
| Research Areas | >>Primary bile acid biosynthesis; / >>Metabolic pathways; / >>PPAR signaling pathway; / >>Cholesterol metabolism |
| Function | Catalytic activity:5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol + NADPH + O(2) = (25R)-5-beta-cholestane-3-alpha,7-alpha,12-alpha,26-tetraol + NADP(+) + H(2)O.,cofactor:Heme group.,Disease:Defects in CYP27A1 are the cause of cerebrotendinous xanthomatosis (CTX) [MIM:213700]. CTX is a rare sterol storage disorder characterized clinically by progressive neurologic dysfunction, premature atherosclerosis, and cataracts.,Function:Catalyzes the first step in the oxidation of the side chain of sterol intermediates; the 27-hydroxylation of 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol. Has also a vitamin D3-25-hydroxylase activity.,pathway:Hormone biosynthesis; cholecalciferol biosynthesis.,similarity:Belongs to the cytochrome P450 family., |
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