Summary

The gene codes for a protein, hydroxysteroid 17-beta dehydrogenase 4. Defects are caused by D-bifunctional protein deficiency (DBPD). A pseudogene is on chromosome 8 [R].


Function

Bifunctional enzyme acting on the peroxisomal beta-oxidation pathway for fatty acids. Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids.

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