HSD17B4

Hydroxysteroid (17-beta) dehydrogenase 4
PDB rendering based on 1ikt.
Available structures PDB Ortholog search: Template:Homologene2PDBe PDBe, Template:Homologene2uniprot RCSB List of PDB id codes 1ikt​, 1s9c​, 1zbq​
Identifiers
Symbols	HSD17B4 ; MFE-2
External IDs	Template:OMIM5 Template:MGI HomoloGene: 358
Gene ontology Molecular function Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Template:GNF GO Cellular component Template:GNF GO Template:GNF GO Biological process Template:GNF GO Template:GNF GO Template:GNF GO
RNA expression pattern
More reference expression data
Orthologs
Template:GNF Ortholog box
Species	Human	Mouse
Entrez	n/a	n/a
Ensembl	n/a	n/a
UniProt	n/a	n/a
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	n/a	n/a
Location (UCSC)	n/a	n/a
PubMed search	n/a	n/a

Hydroxysteroid (17-beta) dehydrogenase 4, also known as HSD17B4, is a human gene.

The HSD17B4 gene encodes an enzyme involved in peroxisomal fatty acid beta-oxidation. It was first identified as a 17-beta-estradiol dehydrogenase (Leenders et al., 1996; van Grunsven et al., 1998). Peroxisomal beta-oxidation of fatty acids, originally described by Lazarow and de Duve (1976), is catalyzed by 3 enzymes: acyl-CoA oxidase (see, e.g., ACOX1, MIM 609751); the 'D-bifunctional enzyme,' with enoyl-CoA-hydratase and D-3-hydroxyacyl-CoA dehydrogenase activity, and 3-ketoacyl-CoA thiolase (MIM 604054). See also the L-bifunctional peroxisomal protein (EHHADH; MIM 607037). The D- and L-bifunctional proteins have different substrate specificities. The D-bifunctional protein catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids and also acts in shortening cholesterol for bile acid formation. In contrast, the L-specific bifunctional protein does not have the latter 2 activities (Jiang et al., 1997).[supplied by OMIM]

Further reading

• de Launoit Y, Adamski J (1999). "Unique multifunctional HSD17B4 gene product: 17beta-hydroxysteroid dehydrogenase 4 and D-3-hydroxyacyl-coenzyme A dehydrogenase/hydratase involved in Zellweger syndrome". J. Mol. Endocrinol. 22 (3): 227–40. PMID 10343282.
• Huyghe S, Mannaerts GP, Baes M, Van Veldhoven PP (2006). "Peroxisomal multifunctional protein-2: the enzyme, the patients and the knockout mouse model". Biochim. Biophys. Acta. 1761 (9): 973–94. doi:10.1016/j.bbalip.2006.04.006. PMID 16766224.CS1 maint: Multiple names: authors list (link)
• Palosaari PM, Hiltunen JK (1990). "Peroxisomal bifunctional protein from rat liver is a trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities". J. Biol. Chem. 265 (5): 2446–9. PMID 2303409.
• Adamski J, Normand T, Leenders F; et al. (1995). "Molecular cloning of a novel widely expressed human 80 kDa 17 beta-hydroxysteroid dehydrogenase IV". Biochem. J. 311 ( Pt 2): 437–43. PMID 7487879.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Markus M, Husen B, Adamski J (1996). "The subcellular localization of 17 beta-hydroxysteroid dehydrogenase type 4 and its interaction with actin". J. Steroid Biochem. Mol. Biol. 55 (5–6): 617–21. PMID 8547189.CS1 maint: Multiple names: authors list (link)
• Jiang LL, Kobayashi A, Matsuura H; et al. (1997). "Purification and properties of human D-3-hydroxyacyl-CoA dehydratase: medium-chain enoyl-CoA hydratase is D-3-hydroxyacyl-CoA dehydratase". J. Biochem. 120 (3): 624–32. PMID 8902629.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Leenders F, Prescher G, Dolez V; et al. (1997). "Assignment of human 17 beta-hydroxysteroid dehydrogenase IV to chromosome 5q2 by fluorescence in situ hybridization". Genomics. 37 (3): 403–4. PMID 8938456.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Jiang LL, Miyazawa S, Souri M, Hashimoto T (1997). "Structure of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein". J. Biochem. 121 (2): 364–9. PMID 9089413.CS1 maint: Multiple names: authors list (link)
• Jiang LL, Kurosawa T, Sato M; et al. (1997). "Physiological role of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein". J. Biochem. 121 (3): 506–13. PMID 9133619.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Novikov D, Dieuaide-Noubhani M, Vermeesch JR; et al. (1997). "The human peroxisomal multifunctional protein involved in bile acid synthesis: activity measurement, deficiency in Zellweger syndrome and chromosome mapping". Biochim. Biophys. Acta. 1360 (3): 229–40. PMID 9197465.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Suzuki Y, Jiang LL, Souri M; et al. (1997). "D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency: a newly identified peroxisomal disorder". Am. J. Hum. Genet. 61 (5): 1153–62. PMID 9345094.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• van Grunsven EG, van Berkel E, Ijlst L; et al. (1998). "Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency". Proc. Natl. Acad. Sci. U.S.A. 95 (5): 2128–33. PMID 9482850.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Dong Y, Qiu QQ, Debear J; et al. (1999). "17Beta-hydroxysteroid dehydrogenases in human bone cells". J. Bone Miner. Res. 13 (10): 1539–46. PMID 9783542.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Leenders F, Dolez V, Begue A; et al. (1999). "Structure of the gene for the human 17beta-hydroxysteroid dehydrogenase type IV". Mamm. Genome. 9 (12): 1036–41. PMID 9880674.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Green VL, Speirs V, Landolt AM; et al. (1999). "17Beta-hydroxysteroid dehydrogenase type 1, 2, 3, and 4 expression and enzyme activity in human anterior pituitary adenomas". J. Clin. Endocrinol. Metab. 84 (4): 1340–5. PMID 10199776.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• van Grunsven EG, Mooijer PA, Aubourg P, Wanders RJ (1999). "Enoyl-CoA hydratase deficiency: identification of a new type of D-bifunctional protein deficiency". Hum. Mol. Genet. 8 (8): 1509–16. PMID 10400999.CS1 maint: Multiple names: authors list (link)
• Itoh M, Suzuki Y, Takashima S (1999). "A novel peroxisomal enzyme, D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein: its expression in the developing human brain". Microsc. Res. Tech. 45 (6): 383–8. doi:10.1002/(SICI)1097-0029(19990615)45:6<383::AID-JEMT5>3.0.CO;2-7. PMID 10402265.CS1 maint: Multiple names: authors list (link)
• Möller G, Leenders F, van Grunsven EG; et al. (1999). "Characterization of the HSD17B4 gene: D-specific multifunctional protein 2/17beta-hydroxysteroid dehydrogenase IV". J. Steroid Biochem. Mol. Biol. 69 (1–6): 441–6. PMID 10419023.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
• Haapalainen AM, van Aalten DM, Meriläinen G; et al. (2001). "Crystal structure of the liganded SCP-2-like domain of human peroxisomal multifunctional enzyme type 2 at 1.75 A resolution". J. Mol. Biol. 313 (5): 1127–38. doi:10.1006/jmbi.2001.5084. PMID 11700068.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)