P3H3 (Q8IVL6) - Overview - Molecular Target Synopsis
P3H3, Prolyl 3-hydroxylase 3
Enzyme Classification 126.96.36.199
Also Known as P3H3_HUMAN, P3H3, LEPREL2
Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils. Required for normal hydroxylation of lysine residues in type I collagen chains in skin, bone, tendon, aorta and cornea. Required for normal skin stability via its role in hydroxylation of lysine residues in collagen alpha chains and in collagen fibril assembly. Apparently not required for normal prolyl 3-hydroxylation on collagen chains, possibly because it functions redundantly with other prolyl 3-hydroxylases. Identified in a complex with PLOD1 and P3H4.
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UniProt: P3H3 is active in the following subcellular-locations: endoplasmic reticulum.
GO terms: P3H3 is active in the following subcellular-locations: catalytic complex, endoplasmic reticulum.