CTX; CP27; CYP27

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

Polyclonal

Rabbit

The antibody detects endogenous levels of total CYP27A1 protein.

Antigen affinity purification.

Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.

1.1 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-CYP27A1 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.

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.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western blotting: 1:200-1:1000
Immunohistochemistry: 1:25-1:100

Gel: 6%SDS-PAGE Lysates (from left to right): Mouse liver and human fetal liver tissue Amount of lysate: 40ug per lane Primary antibody: 1/200 dilution Secondary antibody dilution: 1/8000 Exposure time: 7 minutes

Immunohistochemical analysis of paraffin-embedded Human liver cancer tissue using at dilution 1/25.

60,235 Da

cytochrome P450, family 27, subfamily A, polypeptide 1

sterol 26-hydroxylase, mitochondrial

Sterol 26-hydroxylase, mitochondrial

CYP27??[Similar Products]

CP27A_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 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]

CYP27A1: 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. Defects in CYP27A1 are the cause of cerebrotendinous xanthomatosis (CTX). CTX is a rare sterol storage disorder characterized clinically by progressive neurologic dysfunction, premature atherosclerosis, and cataracts. Belongs to the cytochrome P450 family.

Protein type: Oxidoreductase; EC 1.14.13.15; Mitochondrial; Lipid Metabolism - primary bile acid biosynthesis

Chromosomal Location of Human Ortholog: 2q35

Cellular Component: mitochondrial matrix; mitochondrial inner membrane

Molecular Function: cholestanetriol 26-monooxygenase activity; iron ion binding; cholesterol 26-hydroxylase activity; heme binding; steroid hydroxylase activity; vitamin D3 25-hydroxylase activity

Biological Process: cholesterol metabolic process; bile acid biosynthetic process; bile acid metabolic process; xenobiotic metabolic process; sterol metabolic process

Disease: Cerebrotendinous Xanthomatosis

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