Gene Symbol: Hsd17b4
Description: hydroxysteroid (17-beta) dehydrogenase 4
Alias: MPF-2, peroxisomal multifunctional enzyme type 2, 17-beta-HSD 4, 17-beta-hydroxysteroid dehydrogenase 4, D-bifunctional protein, DBP, MFE-2, multifunctional protein 2, peroxisomal multifunctional enzyme type II
Species: rat
Products:     Hsd17b4

Top Publications

  1. Huyghe S, Schmalbruch H, De Gendt K, Verhoeven G, Guillou F, Van Veldhoven P, et al. Peroxisomal multifunctional protein 2 is essential for lipid homeostasis in Sertoli cells and male fertility in mice. Endocrinology. 2006;147:2228-36 pubmed
    ..In conclusion, the present data demonstrate that peroxisomal beta-oxidation is essential for lipid homeostasis in the testis and for male fertility. ..
  2. Shi R, Zhao C, Zhu H, Yang Y, Wang S, Jiang L. [Relationship between the increase of hepatic D-bifunctional protein activity and bile acid biosynthesis in rats]. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2005;27:321-4 pubmed
    To determine the physiological role of D-bifunctional protein (DBP) in bile acid biosynthesis through investigating the effect of increasing activity of DBP on bile acid biosynthesis...
  3. Zha S, Ferdinandusse S, Hicks J, Denis S, Dunn T, Wanders R, et al. Peroxisomal branched chain fatty acid beta-oxidation pathway is upregulated in prostate cancer. Prostate. 2005;63:316-23 pubmed
    ..downstream from AMACR in the peroxisomal branched chain fatty acid beta-oxidation pathway-D-bifunctional protein (DBP)-is also upregulated in prostate cancer at both mRNA and protein levels, accompanied by increased enzymatic ..
  4. Suzuki Y, Jiang L, Souri M, Miyazawa S, Fukuda S, Zhang Z, et al. D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency: a newly identified peroxisomal disorder. Am J Hum Genet. 1997;61:1153-62 pubmed
    ..This seems to be the first report of D-bifunctional protein deficiency. Patients previously diagnosed as cases of L-bifunctional protein deficiency probably should be reexamined for a possible d-bifunctional protein deficiency. ..
  5. Haapalainen A, Koski M, Qin Y, Hiltunen J, Glumoff T. Binary structure of the two-domain (3R)-hydroxyacyl-CoA dehydrogenase from rat peroxisomal multifunctional enzyme type 2 at 2.38 A resolution. Structure. 2003;11:87-97 pubmed
    The crystal structure of (3R)-hydroxyacyl-CoA dehydrogenase of rat peroxisomal multifunctional enzyme type 2 (MFE-2) was solved at 2.38 A resolution...
  6. Bjelic M, Stojkov N, Radovic S, Baburski A, Janjic M, Kostic T, et al. Prolonged in vivo administration of testosterone-enanthate, the widely used and abused anabolic androgenic steroid, disturbs prolactin and cAMP signaling in Leydig cells of adult rats. J Steroid Biochem Mol Biol. 2015;149:58-69 pubmed publisher
    ..Oppositely, Hsd3b1/2, Hsd3b5, Hsd17b4, Ar, Arr19 increased...
  7. Ferdinandusse S, Ylianttila M, Gloerich J, Koski M, Oostheim W, Waterham H, et al. Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis. Am J Hum Genet. 2006;78:112-24 pubmed
    D-bifunctional protein (DBP) deficiency is an autosomal recessive inborn error of peroxisomal fatty acid oxidation...
  8. Koski K, Haapalainen A, Hiltunen J, Glumoff T. Crystal structure of 2-enoyl-CoA hydratase 2 from human peroxisomal multifunctional enzyme type 2. J Mol Biol. 2005;345:1157-69 pubmed
    2-Enoyl-CoA hydratase 2 is the middle part of the mammalian peroxisomal multifunctional enzyme type 2 (MFE-2), which is known to be important in the beta-oxidation of very-long-chain and alpha-methyl-branched fatty acids as well as in ..
  9. Maxwell M, Bjorkman J, Nguyen T, Sharp P, Finnie J, Paterson C, et al. Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotype. Mol Cell Biol. 2003;23:5947-57 pubmed
    ..The brains of these animals showed disordered lamination in the cerebral cortex, consistent with a neuronal migration defect. Thus, Pex13(-/-) mice reproduce many of the features of Zellweger syndrome and PEX13 deficiency in humans. ..

More Information


  1. Corton J, Bocos C, Moreno E, Merritt A, Cattley R, Gustafsson J. Peroxisome proliferators alter the expression of estrogen-metabolizing enzymes. Biochimie. 1997;79:151-62 pubmed
    ..in rat liver at different treatment times and concentrations of gemfibrozil (GEM) and di-n-butyl phthalate (DBP) were almost identical, suggesting that HSD IV mRNA induction involves the peroxisome proliferator-activated ..
  2. Kashiwayama Y, Tomohiro T, Narita K, Suzumura M, Glumoff T, Hiltunen J, et al. Identification of a substrate-binding site in a peroxisomal beta-oxidation enzyme by photoaffinity labeling with a novel palmitoyl derivative. J Biol Chem. 2010;285:26315-25 pubmed publisher
    ..Mass spectrometric analysis identified the 80-kDa protein as peroxisomal multifunctional enzyme type 2 (MFE2), one of the peroxisomal beta-oxidation enzymes...
  3. van Grunsven E, Mooijer P, Aubourg P, Wanders R. Enoyl-CoA hydratase deficiency: identification of a new type of D-bifunctional protein deficiency. Hum Mol Genet. 1999;8:1509-16 pubmed
  4. Liang M, Lee N, Wang H, Greene A, Kwitek A, Kaldunski M, et al. Molecular networks in Dahl salt-sensitive hypertension based on transcriptome analysis of a panel of consomic rats. Physiol Genomics. 2008;34:54-64 pubmed publisher
    ..Likewise, Acaa2, B4galt6, Colec12, Hsd17b4, and five other ESTs located on chromosome 18 exhibited expression patterns unique to SS-18(BN)...
  5. van Grunsven E, van Berkel E, Ijlst L, Vreken P, de Klerk J, Adamski J, et al. 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. 1998;95:2128-33 pubmed
    ..The results show that the newly identified D-bifunctional protein plays an essential role in the peroxisomal beta-oxidation pathway that cannot be compensated for by the L-specific bifunctional protein. ..
  6. Adamski J, Normand T, Leenders F, Monte D, Begue A, Stehelin D, et al. Molecular cloning of a novel widely expressed human 80 kDa 17 beta-hydroxysteroid dehydrogenase IV. Biochem J. 1995;311 ( Pt 2):437-43 pubmed
    ..0 kb, present in many tissues with highest concentrations in liver, heart, prostate and testes. When over-expressed in mammalian cells, the human 17 beta-HSD IV enzyme displays a specific unidirectional oxidative 17 beta-HSD activity. ..
  7. Corton J, Bocos C, Moreno E, Merritt A, Marsman D, Sausen P, et al. Rat 17 beta-hydroxysteroid dehydrogenase type IV is a novel peroxisome proliferator-inducible gene. Mol Pharmacol. 1996;50:1157-66 pubmed
    ..Increased conversion of estradiol to the less-active estrone by HSD IV induction may explain how phthalate exposure leads to decreases in serum estradiol levels and suppression of ovulation. ..
  8. Jiang L, Miyazawa S, Hashimoto T. Purification and properties of rat D-3-hydroxyacyl-CoA dehydratase: D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. J Biochem. 1996;120:633-41 pubmed
    ..Thus, it is proposed that this enzyme is D-3-hydroxyacyl-CoA dehydratase/ D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. ..
  9. Sokanovic S, Janjic M, Stojkov N, Baburski A, Bjelic M, Andric S, et al. Age related changes of cAMP and MAPK signaling in Leydig cells of Wistar rats. Exp Gerontol. 2014;58:19-29 pubmed publisher
    ..gene expression (decreased Cyp11a1, Cyp17a1 in 12-month-old rats followed by decreased Star/StAR, Hsd3b/HSD3B, Hsd17b4, and increased Cyp19a1 later in life)...
  10. Lovekamp Swan T, Jetten A, Davis B. Dual activation of PPARalpha and PPARgamma by mono-(2-ethylhexyl) phthalate in rat ovarian granulosa cells. Mol Cell Endocrinol. 2003;201:133-41 pubmed
    ..These data support a model in which MEHP activates both PPARalpha and PPARgamma to suppress aromatase and alter other genes related to metabolism and differentiation in the granulosa cell. ..
  11. Dirkx R, Meyhi E, Asselberghs S, Reddy J, Baes M, Van Veldhoven P. Beta-oxidation in hepatocyte cultures from mice with peroxisomal gene knockouts. Biochem Biophys Res Commun. 2007;357:718-23 pubmed
    ..More surprisingly, dicarboxylic fatty acid oxidation was impaired in MFP-1 but not in MFP-2 knockout hepatocytes, indicating that MFP-1 might play more than an obsolete role in peroxisomal beta-oxidation. ..
  12. Dinkel K, Rickert M, Moller G, Adamski J, Meinck H, Richter W. Stiff-man syndrome: identification of 17 beta-hydroxysteroid dehydrogenase type 4 as a novel 80-kDa antineuronal antigen. J Neuroimmunol. 2002;130:184-93 pubmed
    ..The corresponding antigen was identified as 17 beta-hydroxysteroid dehydrogenase type 4 and may represent a possible novel target of autoimmunity in SMS. ..
  13. Jiang L, Miyazawa S, Souri M, Hashimoto T. Structure of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. J Biochem. 1997;121:364-9 pubmed
    ..The cloned cDNA was identical to the human 17 beta-hydroxysteroid dehydrogenase IV cDNA. ..
  14. Kurosawa T, Sato M, Yoshimura T, Jiang L, Hashimoto T, Tohma M. Stereospecific formation of (24R,25R)-3 alpha,7 alpha,12 alpha,24-tetrahydroxy-5 beta-cholestan-26-oic acid catalyzed with a peroxisomal bifunctional D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase. Biol Pharm Bull. 1997;20:295-7 pubmed
    ..These results showed that D-bifunctional protein catalyzes stereospecifically the hydration and dehydration step in bile acid biosynthesis. ..
  15. Dieuaide Noubhani M, Novikov D, Baumgart E, Vanhooren J, Fransen M, Goethals M, et al. Further characterization of the peroxisomal 3-hydroxyacyl-CoA dehydrogenases from rat liver. Relationship between the different dehydrogenases and evidence that fatty acids and the C27 bile acids di- and tri-hydroxycoprostanic acids are metabolized b. Eur J Biochem. 1996;240:660-6 pubmed
    ..L-3-hydroxyacyl-CoA dehydrogenase and delta 3, delta 2-enoyl-CoA isomerase activity (enzyme IV) and (b) multifunctional protein 2 (MFP-2) displaying 2-enoyl-CoA hydratase and D-3-hydroxyacyl-CoA dehydrogenase activity (enzyme III)...