|
|
| Line 1: |
Line 1: |
| {{enzyme
| | Her name is Felicidad Ahmad. Her family members life in Idaho. To maintain birds is 1 of the things he enjoys most. I am a production and distribution officer.<br><br>Here is my page: [http://Ganafc.com/xe/link/2514287 http://Ganafc.com] |
| | EC_number = 1.1.1.145
| |
| | IUBMB_EC_number = 1/1/1/145
| |
| | CAS_number= 9044-85-3
| |
| | Name = 3β-hydroxysteroid dehydrogenase/Δ-5-4 isomerase
| |
| | GO_code = 0003854
| |
| | image =
| |
| | width =
| |
| | caption =
| |
| }}
| |
| {{infobox protein
| |
| | Name = [[HSD3B1|hydroxy-Δ-5-steroid dehydrogenase,<br/>3β- and steroid Δ-isomerase 1]]
| |
| | caption =
| |
| | image =
| |
| | width =
| |
| | HGNCid = 5217
| |
| | Symbol = [[HSD3B1]]
| |
| | AltSymbols = HSDB3, HSD3B
| |
| | EntrezGene = 3283
| |
| | OMIM = 109715
| |
| | RefSeq = NM_000862
| |
| | UniProt = P14060
| |
| | PDB =
| |
| | ECnumber = 1.1.1.145
| |
| | Chromosome = 1
| |
| | Arm = p
| |
| | Band = 13
| |
| | LocusSupplementaryData = -p11
| |
| }}
| |
| {{infobox protein
| |
| | Name = [[HSD3B2|hydroxy-Δ-5-steroid dehydrogenase,<br/>3β- and steroid Δ-isomerase 2]]
| |
| | caption =
| |
| | image =
| |
| | width =
| |
| | HGNCid = 5218
| |
| | Symbol = [[HSD3B2]]
| |
| | AltSymbols =
| |
| | EntrezGene = 3284
| |
| | OMIM = 613890
| |
| | RefSeq = NM_000198
| |
| | UniProt = P26439
| |
| | PDB =
| |
| | ECnumber = 1.1.1.145
| |
| | Chromosome = 1
| |
| | Arm = p
| |
| | Band = 13.1
| |
| | LocusSupplementaryData =
| |
| }}
| |
| '''3-β-HSD''' (or '''3-β-hydroxysteroid dehydrogenase/Δ-5-4 isomerase''') ({{EC number|1.1.1.145}}) is an [[enzyme]] that [[catalysis|catalyzes]] the synthesis of [[progesterone]] from [[pregnenolone]], 17-hydroxyprogesterone from 17-hydroxypregnenolone, and [[androstenedione]] from [[dehydroepiandrosterone]] in the adrenal gland. It is the only enzyme in the adrenal pathway of [[corticosteroid]] synthesis that is not a member of the [[Cytochrome P450]] family.<ref name="pmid3088022">{{cite journal | author = Cravioto MD, Ulloa-Aguirre A, Bermudez JA, Herrera J, Lisker R, Mendez JP, Perez-Palacios G | title = A new inherited variant of the 3 beta-hydroxysteroid dehydrogenase-isomerase deficiency syndrome: evidence for the existence of two isoenzymes | journal = J. Clin. Endocrinol. Metab. | volume = 63 | issue = 2 | pages = 360–7 |date=August 1986 | pmid = 3088022 | doi = 10.1210/jcem-63-2-360| url = }}</ref> In humans, there are two 3-β-HSD [[isozyme]]s encoded by the [[HSD3B1]] and [[HSD3B2]] genes.
| |
| | |
| It is also known as delta 5-delta 4-isomerase, which catalyzes the oxidative conversion of delta 5-3 beta- [[hydroxysteroid]]s to the delta 4-3-keto configuration and is, therefore, essential for the [[biosynthesis]] of all classes of [[hormonal]] [[steroid]]s, namely [[progesterone]], [[glucocorticoids]], [[mineralocorticoids]], [[androgen]]s, and [[estrogen]]s.<ref name="pmid1737804">{{cite journal | author = Lachance Y, Luu-The V, Labrie C, Simard J, Dumont M, de Launoit Y, Guérin S, Leblanc G, Labrie F | title = Characterization of human 3 beta-hydroxysteroid dehydrogenase/delta 5-delta 4-isomerase gene and its expression in mammalian cells | journal = J. Biol. Chem. | volume = 267 | issue = 5 | pages = 3551 |date=February 1992 | pmid = 1737804 | doi = | url = }}</ref>
| |
| The 3-beta HSD complex is responsible for the conversion of:
| |
| | |
| * [[pregnenolone]] to [[progesterone]]
| |
| * 17-alpha-pregnenolone to 17-alpha-progesterone
| |
| * dehydroepiandrosterone ([[DHEA]]) to [[androstenedione]]
| |
| * [[androstenediol]] to [[testosterone]]
| |
| | |
| == Reaction ==
| |
| | |
| 3-β-HSD belongs to the family of [[oxidoreductase]]s, to be specific, those acting on the CH-OH group of donor with NAD<sup>+</sup> or NADP<sup>+</sup> as acceptor. This enzyme participates in c21-steroid hormone metabolism and androgen and estrogen metabolism.
| |
| | |
| 3-β-HSD [[catalysis|catalyzes]] the [[chemical reaction]]:
| |
| | |
| :a 3β-hydroxy-Δ5-steroid + NAD<sup>+</sup> <math>\rightleftharpoons</math> a 3-oxo-Δ5-steroid + NADH + H<sup>+</sup>
| |
| | |
| Thus, the two [[substrate (biochemistry)|substrates]] of this enzyme are 3β-hydroxy-Δ5-steroid and [[nicotinamide adenine dinucleotide|NAD<sup>+</sup>]], whereas its 3 [[product (chemistry)|products]] are 3-oxo-Δ5-steroid, [[nicotinamide adenine dinucleotide|NADH]], and [[hydrogen ion|H<sup>+</sup>]].
| |
| | |
| == Isozymes ==
| |
| | |
| Humans express two 3-β-HSD [[isozyme]]s, [[HSD3B1]] (type I) and [[HSD3B2]] (type II).<ref name="pmid15632317">{{cite journal | author = Simard J, Ricketts ML, Gingras S, Soucy P, Feltus FA, Melner MH | title = Molecular biology of the 3beta-hydroxysteroid dehydrogenase/delta5-delta4 isomerase gene family | journal = Endocr. Rev. | volume = 26 | issue = 4 | pages = 525–82 |date=June 2005 | pmid = 15632317 | doi = 10.1210/er.2002-0050 | url = }}</ref> The type I isoenzyme is expressed in placenta and peripheral tissues, whereas the type II 3β-HSD isoenzyme is expressed in the adrenal gland, ovary, and testis.
| |
| | |
| == Nomenclature ==
| |
| | |
| The systematic name of this enzyme class is '''3β-hydroxy-Δ5-steroid:NAD<sup>+</sup> 3-oxidoreductase'''. Other names in common use include:
| |
| | |
| * progesterone reductase
| |
| * Δ5-3β-hydroxysteroid dehydrogenase
| |
| * 3β-hydroxy-5-ene steroid dehydrogenase
| |
| * 3β-hydroxy steroid dehydrogenase/isomerase
| |
| * 3β-hydroxy-Δ5-C27-steroid dehydrogenase/isomerase
| |
| * 3β-hydroxy-Δ5-C27-steroid oxidoreductase
| |
| * 3β-hydroxy-5-ene-steroid oxidoreductase
| |
| * steroid-Δ5-3β-ol dehydrogenase
| |
| * 3β-HSDH
| |
| * 5-ene-3β-hydroxysteroid dehydrogenase
| |
| * 3β-hydroxy-5-ene-steroid dehydrogenase
| |
| | |
| == Inhibitors ==
| |
| | |
| 3-β-HSD is inhibited by [[trilostane]].<ref name="pmid8809191">{{cite journal | author = Cooke GM | title = Differential effects of trilostane and cyanoketone on the 3 beta-hydroxysteroid dehydrogenase-isomerase reactions in androgen and 16-androstene biosynthetic pathways in the pig testis | journal = J. Steroid Biochem. Mol. Biol. | volume = 58 | issue = 1 | pages = 95–101 |date=April 1996 | pmid = 8809191 |doi = 10.1016/0960-0760(96)00002-7| url = }}</ref>
| |
| | |
| == Biosynthetic pathway ==
| |
| {{Gallery
| |
| |title=
| |
| |footer=
| |
| |width=300
| |
| |lines=3
| |
| |File:Steroidogenesis.svg|Human [[steroidogenesis]], showing reactions of 3β-HSD near-left in green box.
| |
| |Image:Corticosteroid-biosynthetic-pathway-rat.png| Corticosteroid biosynthetic pathway in the rat, showing reaction catalyzed by 3β-HSD (second arrow from the top).
| |
| }}
| |
| | |
| ==Clinical significance==
| |
| A deficiency in the type II form through mutations in HSD3B2 is responsible for a rare form of [[congenital adrenal hyperplasia due to 3 beta-hydroxysteroid dehydrogenase deficiency|congenital adrenal hyperplasia]].<ref name="pmid1363812">{{cite journal | author = Rhéaume E, Simard J, Morel Y, Mebarki F, Zachmann M, Forest MG, New MI, Labrie F | title = Congenital adrenal hyperplasia due to point mutations in the type II 3 beta-hydroxysteroid dehydrogenase gene | journal = Nat. Genet. | volume = 1 | issue = 4 | pages = 239–45 |date=July 1992 | pmid = 1363812 | doi = 10.1038/ng0792-239 | url = }}</ref> No human condition has yet been linked to a deficiency in the type I enzyme. Its importance in placental progesterone production expression suggests that such a mutation would be embryonically lethal.
| |
| | |
| == See also ==
| |
| * [[3α-Hydroxysteroid dehydrogenase]] (3α-HSD)
| |
| | |
| ==References==
| |
| {{reflist|2}}
| |
| | |
| ==Further reading==
| |
| {{refbegin | 2}}
| |
| | |
| * {{cite journal | author = Cheatum SG, Watten JC | year = 1966 | title = Purification and properties of 3-beta-hydroxysteroid dehydrogenase and delta-5-3-ketosteroid isomerase from bovine corpora lutea | journal = Biochim. Biophys. Acta. | volume = 122 | pages = 1–13 | pmid = 4226148 | issue = 1 }}
| |
| * {{cite journal | author = Koritz SB | year = 1964 | title = The conversion of prepnenolone to progesterone by small particle from rat adrenal | journal = Biochemistry | volume = 3 | pages = 1098–1102 | doi = 10.1021/bi00896a015 | pmid=14220672 | issue = 8}}
| |
| * {{cite journal | author = Neville AM, Orr, JC and Engel LL | year = 1968 | title = Delta5-3beta-Hydroxy steroid dehydrogenase activities of bovine adrenal cortex | journal = Biochem. J. | volume = 107 | pages = 20 }}
| |
| {{refend}}
| |
| | |
| {{Alcohol oxidoreductases}}
| |
| {{Steroid metabolism enymes}}
| |
| | |
| [[Category:EC 1.1.1]]
| |
| [[Category:NADH-dependent enzymes]]
| |
| [[Category:Enzymes of unknown structure]]
| |
Her name is Felicidad Ahmad. Her family members life in Idaho. To maintain birds is 1 of the things he enjoys most. I am a production and distribution officer.
Here is my page: http://Ganafc.com