CYP7B1; CP7B; cytochrome P450, family 7, subfamily B, polypeptide 1; oxysterol 7alpha-hydroxylase; cytochrome P450, subfamily VIIB (oxysterol 7 alpha-hydroxylase), polypeptide 1; CBAS3; SPG5A; CYP7B1 antibody; CP7B antibody; cytochrome P450; family 7; subfamily B; polypeptide 1 antibody; oxysterol 7alpha-hydroxylase antibody; cytochrome P450; subfamily VIIB (oxysterol 7 alpha-hydroxylase); polypeptide 1 antibody; CBAS3 antibody; SPG5A antibody

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

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-CYP7B1 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.

Peptide ELISA (EIA), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 128000.
Immunohistochemistry: In paraffin embedded Human Breast shows textured cytoplasm staining in ductal epithelial cells. Recommended concentration, 3-6ug/ml.
Western Blot: Preliminary experiments in Human Liver, Kidney and Prostate lysates gave no specific signal but low background (at antibody concentration up to 1ug/ml).

(4ug/ml) staining of paraffin embedded Human Breast. Steamed antigen retrieval with citrate buffer pH 6, HRP-staining.

58,256 Da

cytochrome P450 family 7 subfamily B member 1

25-hydroxycholesterol 7-alpha-hydroxylase

25-hydroxycholesterol 7-alpha-hydroxylase

CP7B1_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 endoplasmic reticulum membrane protein catalyzes the first reaction in the cholesterol catabolic pathway of extrahepatic tissues, which converts cholesterol to bile acids. This enzyme likely plays a minor role in total bile acid synthesis, but may also be involved in the development of atherosclerosis, neurosteroid metabolism and sex hormone synthesis. Mutations in this gene have been associated with hereditary spastic paraplegia (SPG5 or HSP), an autosomal recessive disorder. [provided by RefSeq, Apr 2016]

CYP7B1: Defects in CYP7B1 are the cause of spastic paraplegia autosomal recessive type 5A (SPG5A). Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. Defects in CYP7B1 are the cause of congenital bile acid synthesis defect type 3 (CBAS3). Clinical features include severe cholestasis, cirrhosis and liver synthetic failure. Hepatic microsomal oxysterol 7-alpha-hydroxylase activity is undetectable. Belongs to the cytochrome P450 family.

Protein type: Endoplasmic reticulum; Lipid Metabolism - primary bile acid biosynthesis; EC 1.14.13.100; Oxidoreductase

Chromosomal Location of Human Ortholog: 8q21.3

Cellular Component: endoplasmic reticulum membrane

Molecular Function: 25-hydroxycholesterol 7alpha-hydroxylase activity; heme binding; iron ion binding; oxysterol 7-alpha-hydroxylase activity

Biological Process: bile acid biosynthetic process; cholesterol metabolic process; negative regulation of estrogen receptor signaling pathway; positive regulation of epithelial cell proliferation; sterol metabolic process

Disease: Bile Acid Synthesis Defect, Congenital, 3; Spastic Paraplegia 5a, Autosomal Recessive

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