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Hsd17b4

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Title: Hsd17b4  
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Subject: Peroxisomal disorder, Sterol carrier protein, 17Beta Hydroxysteroid dehydrogenase, D-bifunctional protein deficiency
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Hsd17b4

Hydroxysteroid (17-beta) dehydrogenase 4
PDB rendering based on 1ikt.
Available structures
PDB Ortholog search: RCSB
Identifiers
1.1.1.n12, 4.2.1.107, 4.2.1.119
RNA expression pattern

Peroxisomal multifunctional enzyme type 2 is a protein that in humans is encoded by the HSD17B4 gene.[1][2][3]

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][3]

See also

References

Further reading

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