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lüll Unique multifunctional HSD17B4 gene product: 17beta-hydroxysteroid dehydrogenase 4 and D-3-hydroxyacyl-coenzyme A dehydrogenase/hydratase involved in Zellweger syndrome de Launoit Y; Adamski JJ Mol Endocrinol 1999[Jun]; 22 (3): 227-40Six types of human 17beta-hydroxysteroid dehydrogenases catalyzing the conversion of estrogens and androgens at position C17 have been identified so far. The peroxisomal 17beta-hydroxysteroid dehydrogenase type 4 (17beta-HSD 4, gene name HSD17B4) catalyzes the oxidation of estradiol with high preference over the reduction of estrone. The highest levels of 17beta-HSD 4 mRNA transcription and specific activity are found in liver and kidney followed by ovary and testes. A 3 kb mRNA codes for an 80 kDa (737 amino acids) protein featuring domains which are not present in the other 17beta-HSDs. The N-terminal domain of 17beta-HSD 4 reveals only 25% amino acid similarity with the other types of 17beta-HSDs. The 80 kDa protein is N-terminally cleaved to a 32 kDa enzymatically active fragment. Both the 80 kDa and the N-terminal 32 kDa (amino acids 1-323) protein are able to perform the dehydrogenase reaction not only with steroids at the C17 position but also with D-3-hydroxyacyl-coenzyme A (CoA). The enzyme is not active with L-stereoisomers. The central part of the 80 kDa protein (amino acids 324-596) catalyzes the 2-enoyl-acyl-CoA hydratase reaction with high efficiency. The C-terminal part of the 80 kDa protein (amino acids 597-737) facilitates the transfer of 7-dehydrocholesterol and phosphatidylcholine between membranes in vitro. The HSD17B4 gene is stimulated by progesterone, and ligands of PPARalpha (peroxisomal proliferator activated receptor alpha) such as clofibrate, and is down-regulated by phorbol esters. Mutations in the HSD17B4 lead to a fatal form of Zellweger syndrome.|*Multienzyme Complexes[MESH]|17-Hydroxysteroid Dehydrogenases/genetics/isolation & purification/*metabolism[MESH]|3-Hydroxyacyl CoA Dehydrogenases/*metabolism[MESH]|Amino Acid Sequence[MESH]|Animals[MESH]|Cloning, Molecular[MESH]|Enoyl-CoA Hydratase/*metabolism[MESH]|Gene Expression Regulation, Enzymologic[MESH]|Humans[MESH]|Hydro-Lyases[MESH]|Molecular Sequence Data[MESH]|Mutation[MESH]|Peroxisomal Multifunctional Protein-2[MESH]|Sequence Homology, Amino Acid[MESH]|Subcellular Fractions/enzymology[MESH]|Swine[MESH]|Zellweger Syndrome/*enzymology[MESH] |