prolyl hydroxylase

(redirected from Prolyl 3-hydroxylase)

pro·lyl hy·drox·y·lase

an enzyme that catalyzes the hydroxylation of certain prolyl residues in collagen precursors using molecular oxygen, ferrous ion, ascorbic acid, and α-ketoglutarate; a vitamin C deficiency directly affects the activity of this enzyme; one form of this enzyme (prolyl 4-hydroxylase) synthesizes 4-hydroxyprolyl residues while another produces 3-hydroxyprolyl residues.
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Prolyl 3-hydroxylase 1 deficiency causes a recessive metabolic bone disorder resembling lethal/severe osteogenesis imperfecta.