DBP; MFE-2; MPF-2; PRLTS1; SDR8C1

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

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

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. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:500 - 1:2000
IHC: 1:50 - 1:200

Western blot analysis of extracts of various cell lines, using HSD17B4 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.

736

hydroxysteroid (17-beta) dehydrogenase 4

peroxisomal multifunctional enzyme type 2; 17-beta-HSD 4; 17-beta-HSD IV; beta-keto-reductase; multifunctional protein 2; beta-hydroxyacyl dehydrogenase; D-3-hydroxyacyl-CoA dehydratase; D-bifunctional protein, peroxisomal; peroxisomal multifunctional protein 2; 17-beta-hydroxysteroid dehydrogenase 4; 17beta-estradiol dehydrogenase type IV; short chain dehydrogenase/reductase family 8C, member 1; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA hydratase

Peroxisomal multifunctional enzyme type 2

EDH17B4; 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 4.2.1.119; EC 4.2.1.107; Cell development/differentiation; Mitochondrial; Oxidoreductase; Lipid Metabolism - primary bile acid biosynthesis; Lyase; EC 1.1.1.n12

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; long-chain-enoyl-CoA hydratase activity; isomerase 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; 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

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