Genomes and Genes
Gene Symbol: Hsd17b4
Description: hydroxysteroid (17-beta) dehydrogenase 4
Alias: 17-beta-HSD, 17[b]-HSD, DBP, MFE-2, MFP2, MPF-2, Mfp-2, perMFE-2, peroxisomal multifunctional enzyme type 2, 17-beta-HSD 4, 17-beta-hydroxysteroid dehydrogenase 4, D-bifunctional protein, multifunctional protein 2
- Jia Y, Qi C, Zhang Z, Hashimoto T, Rao M, Huyghe S, et al. Overexpression of peroxisome proliferator-activated receptor-alpha (PPARalpha)-regulated genes in liver in the absence of peroxisome proliferation in mice deficient in both L- and D-forms of enoyl-CoA hydratase/dehydrogenase enzymes of peroxisomal be. J Biol Chem. 2003;278:47232-9 pubmed
- Verheijden S, Bottelbergs A, Krysko O, Krysko D, Beckers L, De Munter S, et al. Peroxisomal multifunctional protein-2 deficiency causes neuroinflammation and degeneration of Purkinje cells independent of very long chain fatty acid accumulation. Neurobiol Dis. 2013;58:258-69 pubmed publisher..the cellular mechanisms of neurodegeneration in these patients, we developed a mouse model lacking multifunctional protein 2 (MFP2, also called D-bifunctional protein), a central enzyme of peroxisomal ?-oxidation, in all neural ..
- Martens K, Ver Loren van Themaat E, van Batenburg M, Heinaniemi M, Huyghe S, Van Hummelen P, et al. Coordinate induction of PPAR alpha and SREBP2 in multifunctional protein 2 deficient mice. Biochim Biophys Acta. 2008;1781:694-702 pubmed publisherMice with inactivation of the D-specific multifunctional protein 2 (MFP2), a crucial enzyme of peroxisomal beta-oxidation, develop multiple pathologies in diverse tissues already starting in the postnatal period...
- Suraweera N, Haines J, McCart A, Rogers P, Latchford A, Coster M, et al. Genetic determinants modulate susceptibility to pregnancy-associated tumourigenesis in a recombinant line of Min mice. Hum Mol Genet. 2006;15:3429-35 pubmed..015). Our results provide the first experimental evidence for genetic determinants controlling pregnancy-associated tumourigenesis; analogous genetic factors may exist in humans. ..
- Huyghe S, Mannaerts G, Baes M, Van Veldhoven P. Peroxisomal multifunctional protein-2: the enzyme, the patients and the knockout mouse model. Biochim Biophys Acta. 2006;1761:973-94 pubmed..In the present review, the identification and biochemical characteristics of MFP-2, and the consequences of MFP-2 dysfunction in humans and in mice will be discussed. ..
- De Munter S, Verheijden S, Vanderstuyft E, Malheiro A, Brites P, Gall D, et al. Early-onset Purkinje cell dysfunction underlies cerebellar ataxia in peroxisomal multifunctional protein-2 deficiency. Neurobiol Dis. 2016;94:157-68 pubmed publisher..At the age of 4weeks, Nestin-Mfp2(-/-) mice showed impaired motor learning on the accelerating rotarod and underperformed on the balance beam test...
- Mustonen M, Poutanen M, Isomaa V, Vihko P, Vihko R. Cloning of mouse 17beta-hydroxysteroid dehydrogenase type 2, and analysing expression of the mRNAs for types 1, 2, 3, 4 and 5 in mouse embryos and adult tissues. Biochem J. 1997;325 ( Pt 1):199-205 pubmed..The data therefore suggest that there is transient oestradiol production in the early days of embryonic development, after which inactivation of sex steroids predominates in the fetus and placenta. ..
- 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. ..
- Hisano T, Tsuge T, Fukui T, Iwata T, Miki K, Doi Y. Crystal structure of the (R)-specific enoyl-CoA hydratase from Aeromonas caviae involved in polyhydroxyalkanoate biosynthesis. J Biol Chem. 2003;278:617-24 pubmed..In addition, this study provides the first structural information of the (R)-hydratase family and may facilitate further functional studies for members of the family. ..
- Huyghe S, Casteels M, Janssen A, Meulders L, Mannaerts G, Declercq P, et al. Prenatal and postnatal development of peroxisomal lipid-metabolizing pathways in the mouse. Biochem J. 2001;353:673-80 pubmed
- Verheijden S, Beckers L, Casazza A, Butovsky O, Mazzone M, Baes M. Identification of a chronic non-neurodegenerative microglia activation state in a mouse model of peroxisomal Î²-oxidation deficiency. Glia. 2015;63:1606-20 pubmed publisher..We previously showed that mice lacking multifunctional protein 2 (MFP2), a pivotal enzyme in peroxisomal Î²-oxidation, persistently accumulate reactive myeloid cells ..
- Kleinecke S, Richert S, de Hoz L, BrÃ¼gger B, Kungl T, Asadollahi E, et al. Peroxisomal dysfunctions cause lysosomal storage and axonal Kv1 channel redistribution in peripheral neuropathy. elife. 2017;6: pubmed publisher..This reveals a new aspect of axon-glia interactions, with Schwann cell lipid metabolism regulating the anchorage of juxtaparanodal Kv1-channels. ..
- 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. ..
- Normand T, Husen B, Leenders F, Pelczar H, Baert J, Begue A, et al. Molecular characterization of mouse 17 beta-hydroxysteroid dehydrogenase IV. J Steroid Biochem Mol Biol. 1995;55:541-8 pubmed..The multitude of enzymes acting at C17 might be necessary for a precise control of hormone levels. ..
- Carstensen J, Tesdorpf J, Kaufmann M, Markus M, Husen B, Leenders F, et al. Characterization of 17 beta-hydroxysteroid dehydrogenase IV. J Endocrinol. 1996;150 Suppl:S3-12 pubmed