HSD17B4

Hydroxysteroid (17-beta) dehydrogenase 4, also known as HSD17B4, is a human gene.cite web | title = Entrez Gene: HSD17B4 hydroxysteroid (17-beta) dehydrogenase 4| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=3295| accessdate = ]

PBB_Summary
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summary_text = 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] cite web | title = Entrez Gene: HSD17B4 hydroxysteroid (17-beta) dehydrogenase 4| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=3295| accessdate = ]

References

Further reading

PBB_Further_reading
citations =
*cite journal | author=de Launoit Y, Adamski J |title=Unique multifunctional HSD17B4 gene product: 17beta-hydroxysteroid dehydrogenase 4 and D-3-hydroxyacyl-coenzyme A dehydrogenase/hydratase involved in Zellweger syndrome. |journal=J. Mol. Endocrinol. |volume=22 |issue= 3 |pages= 227–40 |year= 1999 |pmid= 10343282 |doi=
*cite journal | author=Huyghe S, Mannaerts GP, Baes M, Van Veldhoven PP |title=Peroxisomal multifunctional protein-2: the enzyme, the patients and the knockout mouse model. |journal=Biochim. Biophys. Acta |volume=1761 |issue= 9 |pages= 973–94 |year= 2006 |pmid= 16766224 |doi= 10.1016/j.bbalip.2006.04.006
*cite journal | author=Palosaari PM, Hiltunen JK |title=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. |journal=J. Biol. Chem. |volume=265 |issue= 5 |pages= 2446–9 |year= 1990 |pmid= 2303409 |doi=
*cite journal | author=Adamski J, Normand T, Leenders F, "et al." |title=Molecular cloning of a novel widely expressed human 80 kDa 17 beta-hydroxysteroid dehydrogenase IV. |journal=Biochem. J. |volume=311 ( Pt 2) |issue= |pages= 437–43 |year= 1995 |pmid= 7487879 |doi=
*cite journal | author=Markus M, Husen B, Adamski J |title=The subcellular localization of 17 beta-hydroxysteroid dehydrogenase type 4 and its interaction with actin. |journal=J. Steroid Biochem. Mol. Biol. |volume=55 |issue= 5-6 |pages= 617–21 |year= 1996 |pmid= 8547189 |doi=
*cite journal | author=Jiang LL, Kobayashi A, Matsuura H, "et al." |title=Purification and properties of human D-3-hydroxyacyl-CoA dehydratase: medium-chain enoyl-CoA hydratase is D-3-hydroxyacyl-CoA dehydratase. |journal=J. Biochem. |volume=120 |issue= 3 |pages= 624–32 |year= 1997 |pmid= 8902629 |doi=
*cite journal | author=Leenders F, Prescher G, Dolez V, "et al." |title=Assignment of human 17 beta-hydroxysteroid dehydrogenase IV to chromosome 5q2 by fluorescence in situ hybridization. |journal=Genomics |volume=37 |issue= 3 |pages= 403–4 |year= 1997 |pmid= 8938456 |doi=
*cite journal | author=Jiang LL, Miyazawa S, Souri M, Hashimoto T |title=Structure of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. |journal=J. Biochem. |volume=121 |issue= 2 |pages= 364–9 |year= 1997 |pmid= 9089413 |doi=
*cite journal | author=Jiang LL, Kurosawa T, Sato M, "et al." |title=Physiological role of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. |journal=J. Biochem. |volume=121 |issue= 3 |pages= 506–13 |year= 1997 |pmid= 9133619 |doi=
*cite journal | author=Novikov D, Dieuaide-Noubhani M, Vermeesch JR, "et al." |title=The human peroxisomal multifunctional protein involved in bile acid synthesis: activity measurement, deficiency in Zellweger syndrome and chromosome mapping. |journal=Biochim. Biophys. Acta |volume=1360 |issue= 3 |pages= 229–40 |year= 1997 |pmid= 9197465 |doi=
*cite journal | author=Suzuki Y, Jiang LL, Souri M, "et al." |title=D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency: a newly identified peroxisomal disorder. |journal=Am. J. Hum. Genet. |volume=61 |issue= 5 |pages= 1153–62 |year= 1997 |pmid= 9345094 |doi=
*cite journal | author=van Grunsven EG, van Berkel E, Ijlst L, "et al." |title=Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=95 |issue= 5 |pages= 2128–33 |year= 1998 |pmid= 9482850 |doi=
*cite journal | author=Dong Y, Qiu QQ, Debear J, "et al." |title=17Beta-hydroxysteroid dehydrogenases in human bone cells. |journal=J. Bone Miner. Res. |volume=13 |issue= 10 |pages= 1539–46 |year= 1999 |pmid= 9783542 |doi=
*cite journal | author=Leenders F, Dolez V, Begue A, "et al." |title=Structure of the gene for the human 17beta-hydroxysteroid dehydrogenase type IV. |journal=Mamm. Genome |volume=9 |issue= 12 |pages= 1036–41 |year= 1999 |pmid= 9880674 |doi=
*cite journal | author=Green VL, Speirs V, Landolt AM, "et al." |title=17Beta-hydroxysteroid dehydrogenase type 1, 2, 3, and 4 expression and enzyme activity in human anterior pituitary adenomas. |journal=J. Clin. Endocrinol. Metab. |volume=84 |issue= 4 |pages= 1340–5 |year= 1999 |pmid= 10199776 |doi=
*cite journal | author=van Grunsven EG, Mooijer PA, Aubourg P, Wanders RJ |title=Enoyl-CoA hydratase deficiency: identification of a new type of D-bifunctional protein deficiency. |journal=Hum. Mol. Genet. |volume=8 |issue= 8 |pages= 1509–16 |year= 1999 |pmid= 10400999 |doi=
*cite journal | author=Itoh M, Suzuki Y, Takashima S |title=A novel peroxisomal enzyme, D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein: its expression in the developing human brain. |journal=Microsc. Res. Tech. |volume=45 |issue= 6 |pages= 383–8 |year= 1999 |pmid= 10402265 |doi= 10.1002/(SICI)1097-0029(19990615)45:6<383::AID-JEMT5>3.0.CO;2-7 |doilabel=10.1002/(SICI)1097-0029(19990615)45:6383::AID-JEMT53.0.CO;2-7
*cite journal | author=Möller G, Leenders F, van Grunsven EG, "et al." |title=Characterization of the HSD17B4 gene: D-specific multifunctional protein 2/17beta-hydroxysteroid dehydrogenase IV. |journal=J. Steroid Biochem. Mol. Biol. |volume=69 |issue= 1-6 |pages= 441–6 |year= 1999 |pmid= 10419023 |doi=
*cite journal | author=Haapalainen AM, van Aalten DM, Meriläinen G, "et al." |title=Crystal structure of the liganded SCP-2-like domain of human peroxisomal multifunctional enzyme type 2 at 1.75 A resolution. |journal=J. Mol. Biol. |volume=313 |issue= 5 |pages= 1127–38 |year= 2001 |pmid= 11700068 |doi= 10.1006/jmbi.2001.5084

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