17b-HSD4 Polyclonal Antibody; HSD17B4; EDH17B4; Peroxisomal multifunctional enzyme type 2; MFE-2; 17-beta-hydroxysteroid dehydrogenase 4; 17-beta-HSD 4; D-bifunctional protein; DBP; Multifunctional protein 2; MPF-2

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

Polyclonal

Rabbit

17b-HSD4 Polyclonal Antibody detects endogenous levels of 17b-HSD4 protein.

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

1 mg/ml (lot specific)

Store at -20 degree C/1 year

Small volumes of anti-17beta-HSD4 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.

Total protein Ab

Western Blot (WB), ELISA (EIA)

Western Blot: 1:500 - 1:2000
ELISA: 1:20000
Not yet tested in other applications.

Western Blot analysis of HuvEc, K562 cells using 17b-HSD4 Polyclonal Antibody.

77,870 Da

hydroxysteroid (17-beta) dehydrogenase 4

peroxisomal multifunctional enzyme type 2

Peroxisomal multifunctional enzyme type 2

EDH17B4; SDR8C1; MFE-2; 17-beta-HSD 4; DBP; MPF-2??[Similar Products]

DHB4_HUMAN

The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). An apparent pseudogene of this gene is present on chromosome 8. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, May 2014]

HSD17B4: Bifunctional enzyme acting on the peroxisomal beta- oxidation pathway for fatty acids. Catalyzes the formation of 3- ketoacyl-CoA intermediates from both straight-chain and 2-methyl- branched-chain fatty acids. Defects in HSD17B4 are a cause of D-bifunctional protein deficiency (DBPD). DBPD is a disorder of peroxisomal fatty acid beta-oxidation. Defects in HSD17B4 are the cause of Perrault syndrome (PRS). PRS is a sex-influenced disorder characterized by sensorineural deafness in both males and females and ovarian dysgenesis in females. Some patients also have neurologic manifestations, including mild mental retardation and cerebellar and peripheral nervous system involvement. Belongs to the short-chain dehydrogenases/reductases (SDR) family.

Protein type: EC 1.1.1.n12; Oxidoreductase; Cell development/differentiation; Lyase; EC 4.2.1.119; Lipid Metabolism - primary bile acid biosynthesis; EC 4.2.1.107; Mitochondrial

Chromosomal Location of Human Ortholog: 5q21

Cellular Component: peroxisomal membrane; peroxisomal matrix; membrane; mitochondrion; intracellular membrane-bound organelle; peroxisome

Molecular Function: protein homodimerization activity; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity; isomerase activity; long-chain-enoyl-CoA hydratase activity; 3-hydroxyacyl-CoA dehydrogenase activity; receptor binding

Biological Process: osteoblast differentiation; estrogen metabolic process; bile acid biosynthetic process; fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid beta-oxidation; very-long-chain fatty acid metabolic process; bile acid metabolic process; metabolic process; unsaturated fatty acid metabolic process; androgen metabolic process; cellular lipid metabolic process; Sertoli cell development

Disease: Perrault Syndrome 1; D-bifunctional Protein Deficiency

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.