Anti-ACYP11A1抗体This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many

产品编号 yb-3608R
英文名称 CAnti-ACYP11A1抗体
中文名称 细胞色素P450 11A1抗体
别    名 Cholesterol 20 22 desmolase; Cholesterol desmolase; Cholesterol monooxygenase (side chain cleaving); Cholesterol side chain cleavage enzyme; CYP 11A; CYP11A; CYPXIA1; Cytochrome P450 11A1; Cytochrome P450 11A1 mitochondrial; Cytochrome P450 family 11 subfamily A polypeptide 1; Cytochrome P450 subfamily XIA (cholesterol side chain cleavage); Cytochrome P450 subfamily XIA; Cytochrome P450C11A1; P450(scc); P450SCC; Steroid 20 22 lyase; CP17A_HUMAN.
Anti-ACYP11A1抗体  
说 明 书 0.1ml  0.2ml  
研究领域 肿瘤  细胞生物  免疫学  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, 
产品应用 WB=1:100-500 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:50-500 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 53/57kDa
细胞定位 细胞浆 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human CYP11A1/P450SCC
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 protein localizes to the mitochondrial inner membrane and catalyzes the conversion of cholesterol to pregnenolone, the first and rate-limiting step in the synthesis of the steroid hormones. Two transcript variants encoding different isoforms have been found for this gene. The cellular location of the smaller isoform is unclear since it lacks the mitochondrial-targeting transit peptide. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone.

Subcellular Location:
Mitochondrion membrane.

DISEASE:
Defects in CYP11A1 are the cause of adrenal insufficiency congenital with 46,XY sex reversal (AICSR) [MIM:613743]. A rare disorder that can present as acute adrenal insufficiency in infancy or childhood. ACTH and plasma renin activity are elevated and adrenal steroids are inappropriay low or absent; the 46,XY patients have female external genitalia, sometimes with clitoromegaly. The phenotypic spectrum ranges from prematurity, complete underandrogenization, and severe early-onset adrenal failure to term birth with clitoromegaly and later-onset adrenal failure. Patients with congenital adrenal insufficiency do not manifest the massive adrenal enlargement typical of congenital lipoid adrenal hyperplasia.

Similarity:
Belongs to the cytochrome P450 family.



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