CYP11A1, Polyclonal Antibody

Cholesterol desmolase; CP11A; CYP 11A; CYP11A; Cyp11a; Cyp11a-1; CYP11A1; CYPXIA1; Cytochrome P450 11A1; Cytochrome P450(scc); P450SCC; P05108 ; Cholesterol side-chain cleavage enzyme, mitochondrial; cytochrome P450, family 11, subfamily A, polypeptide 1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Immunogen affinity purified.

Lyophilized

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-CYP11A1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit IgG polyclonal antibody for Cholesterol side-chain cleavage enzyme, mitochondrial (CYP11A1) detection.
Background: CYP11A1 (Cytochrome p450, family 11, subfamily A, polypeptide 1), commonly referred to as P450scc, is a mitochondrial enzyme associated with the conversion of cholesterol to pregnenolone. CYP11A1 is a member of the cytochrome P450 superfamily of enzymes. The CYP11A1 gene is mapped on 15q24.1. Expression of the CYP11A1 gene may play a role in skin physiology and pathology and that cutaneous proopiomelanocortin activity may be autoregulated by a feedback mechanism involving glucocorticoids synthesized locally. Using in vitro studies, CYP11A1 catalyzed the side-chain cleavage of 7-dehydrocholesterol to form 7-dehydropregnenolone. In addition, CYP11A1 catalyzed the metabolism of biologically inert vitamin D3, which is formed from 7-dehydrocholesterol, to form 2 hydroxylated products, 20-hydroxyvitamin D3 and 20, 22-dihydroxyvitamin D3. Mutations in the CYP11A1 gene cause congenital adrenal insufficiency with partial or complete 46, XY sex reversal.

Western Blot (WB)

Western Blot: 0.1-0.5mug/ml; Tested Species: Human, Rat
Tested Species:In-house tested species with positive results.
Other applications have not been tested.

Western blot analysis of CYP11A1 expression in rat brain extract (lane 1), rat kidney extract (lane 2) and 293T whole cell lysates (lane 3). CYP11A1 at 75KD was detected using rabbit anti-CYP11A1 Antigen Affinity purified polyclonal antibody at 0.5 ug/mL. The blot was developed using chemiluminescence (ECL) method.

42,152 Da

cytochrome P450 family 11 subfamily A member 1

cholesterol side-chain cleavage enzyme, mitochondrial

Cholesterol side-chain cleavage enzyme, mitochondrial

CYP11A  [Similar Products]

CP11A_HUMAN

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]

CYP11A1: Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone. Defects in CYP11A1 are the cause of adrenal insufficiency congenital with 46,XY sex reversal (AICSR). 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 inappropriately 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. Belongs to the cytochrome P450 family.

Protein type: EC 1.14.15.6; Oxidoreductase; Mitochondrial; Lipid Metabolism - C21-steroid hormone

Chromosomal Location of Human Ortholog: 15q23-q24

Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion

Molecular Function: cholesterol monooxygenase (side-chain-cleaving) activity; heme binding; oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NADH or NADPH as one donor, and incorporation of one atom of oxygen

Biological Process: C21-steroid hormone biosynthetic process; cholesterol metabolic process; glucocorticoid biosynthetic process; sterol metabolic process; vitamin D metabolic process

Disease: Adrenal Insufficiency, Congenital, With 46,xy Sex Reversal, Partial Or Complete

1. al Kandari, H., Katsumata, N., Alexander, S., Rasoul, M. A. Homozygous mutation of P450 side-chain cleavage enzyme gene (CYP11A1) in 46,XY patient with adrenal insufficiency, complete sex reversal, and agenesis of corpus callosum. J. Clin. Endocr. Metab. 91: 2821-2826, 2006.
2. Chung, B.-C., Matteson, K. J., Voutilainen, R., Mohandas, T. K., Miller, W. L. Human cholesterol side-chain cleavage enzyme, P450scc: cDNA cloning, assignment of the gene to chromosome 15, and expression in the placenta. Proc. Nat. Acad. Sci. 83: 8962-8966, 1986.
3. Guryev, O., Carvalho, R. A., Usanov, S., Gilep, A., Estabrook, R. W. A pathway for the metabolism of vitamin D3: unique hydroxylated metabolites formed during catalysis with cytochrome P450scc (CYP11A1). Proc. Nat. Acad. Sci. 100: 14754-14759, 2003.

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