产品名称
CYP27A1 Rabbit Polyclonal Antibody
别名
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
蛋白名称
Sterol 26-hydroxylase mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1593
Human Swissprot No.
Q02318
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q02318/entry
Mouse Swissprot No.
Q9DBG1
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9DBG1
免疫原
The antiserum was produced against synthesized peptide derived from human Cytochrome P450 27A1. AA range:101-150
特异性
CYP27A1 Polyclonal Antibody detects endogenous levels of CYP27A1 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
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).
细胞定位
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. .
信号通路
Primary bile acid biosynthesis;PPAR;
功能
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
