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PLOD3 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.
Gene Name: | procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3 |
Synonyms: | PLOD3, LH3, Lysine hydroxylase 3, Lysyl hydroxlase 3, Lysyl hydroxylase 3 |
Target Sequences: | NM_001084 NP_001075.1 O60568 |
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