The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VIB have deficiencies in lysyl hydroxylase activity.
Pathway
Lysine degradation, organism-specific biosystem; Lysine degradation, conserved biosystem; Other types of O-glycan biosynthesis, organism-specific biosystem; Other types of O-glycan biosynthesis, conserved biosystem.
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