MFE-2; 17-beta-hydroxysteroid dehydrogenase 4; 17-beta-HSD 4; D-bifunctional protein; DBP; Multifunctional protein 2; MPF-2; HSD17B4; EDH17B4

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

Polyclonal

IgG

Rabbit

Antigen Affinity Purified

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

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

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.

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.

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

Recommended dilution: WB:1:200-1000
IHC:1:20-1:200

All lanes: Peroxisomal multifunctional enzyme type 2 antibody at 3ug/ml+mouse liver tissue
Secondary
Goat polyclonal to rabbit at 1/10000 dilution
Predicted band size: 80,84,78 kDa
Observed band size: 80 kDa

Immunohistochemistry of paraffin-embedded human liver using MBS1497952 at dilution 1:100

Immunohistochemistry of paraffin-embedded human epityphlon using MBS1497952 at dilution 1:100

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

Chromosomal Location of Human Ortholog: 5q21

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

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

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

Disease: D-bifunctional Protein Deficiency; Perrault Syndrome 1

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