Anti-CYP27A1抗体This gene encodes a member of the cytochrome P450 superfamily of enzymes.

产品编号 yb-5049R
英文名称 Anti-CYP27A1抗体
中文名称 *27α羟化酶抗体
别    名 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 26-hydroxylase; 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol 27-hydroxylase; 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 27-hydroxylase; Cholestanetriol 26 monooxygenase; CP27; CTX; CYP; CYP27; Cytochrome P 450C27/25; Cytochrome P450 27; Cytochrome P450 27 mitochondrial; Cytochrome P450 family 27 subfamily A polypeptide 1; CP27A_HUMAN; Cytochrome P450 subfamily XXVIIA (steroid 27-hydroxylase cerebrotendinous xanthomatosis) polypeptide 1; Sterol 26 hydroxylase; Sterol 26 hydroxylase mitochondrial; Sterol 27 hydroxylase; Vitamin D(3) 25 hydroxylase.
Anti-CYP27A1抗体    
说 明 书 0.2ml  
研究领域 肿瘤  心血管  细胞生物  免疫学  染色质和核信号  信号转导  脂蛋白  新陈代谢  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 57kDa
细胞定位 细胞浆 细胞膜 线粒体
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CYP27A1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
产品介绍 background:
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].

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. 

Subcellular Location:
Mitochondrion membrane. 

DISEASE:
Cerebrotendinous xanthomatosis (CTX) [MIM:213700]: Rare sterol storage disorder characterized clinically by progressive neurologic dysfunction, premature atherosclerosis, and cataracts. Note=The disease is caused by mutations affecting the gene represented in this entry. 

Similarity:
Belongs to the cytochrome P450 family.

Database links:
UniProtKB/Swiss-Prot: Q02318.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.