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; (3R)-hydroxyacyl-CoA dehydrogenase; 111n12; Enoyl-CoA hydratase 2; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA hydratase; HSD17B4; EDH17B4

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

Polyclonal

Rabbit Ig

Rabbit

This HSD17B4 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 341-370 amino acids from the Central region of human HSD17B4.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.25 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-HSD17B4 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.

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. [provided by
RefSeq].

Cancer; Cardiovascular; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

HSD17B4 Antibody (Center) western blot analysis in MCF-7,ZR-75-1,HepG2 cell line lysates (35ug/lane).This demonstrates the HSD17B4 antibody detected the HSD17B4 protein (arrow).

HSD17B4 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human liver tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of HSD17B4 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

79686

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

Chromosomal Location of Human Ortholog: 5q21

Cellular Component: peroxisomal membrane; peroxisomal matrix; intracellular membrane-bound organelle; membrane; mitochondrion; 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; metabolic process; very-long-chain fatty acid metabolic process; bile acid 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

Canzian, F., et al. Hum. Mol. Genet. 19(19):3873-3884(2010)
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Kashiwayama, Y., et al. J. Biol. Chem. 285(34):26315-26325(2010)
Pierce, S.B., et al. Am. J. Hum. Genet. 87(2):282-288(2010)
Liu, C.Y., et al. Carcinogenesis 31(7):1259-1263(2010)

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