Lysyl hydroxylase
From Wikipedia, the free encyclopedia
Lysyl hydroxylase (or procollagen-lysine 5-dioxygenase) is an oxygenase enzyme that catalyzes the hydroxylation of lysine to hydroxylysine. This reaction is necessary to the formation and stabilization of collagen. It takes place following protein synthesis (as a post-translational modification). The protein is a membrane-bound homodimeric enzyme that is localized to the cisternae (lumen) of the rough endoplasmic reticulum.
It requires iron and vitamin C as cofactors.
[edit] Pathology
A deficiency is associated with Ehlers-Danlos syndrome (type 6).
[edit] External links
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Fibril forming
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FACIT: type IX (COL9A1, COL9A2, COL9A3) · type XII (COL12A1) · COL14A1 · COL16A1 · COL19A1 · COL20A1 · COL21A1 · COL22A1
basement membrane: type IV (COL4A1, COL4A2, COL4A3, COL4A4, COL4A5, COL4A6)
multiplexin: COL15A1 · type XVIII (COL18A1, Endostatin)
transmembrane: COL13A1 · COL17A1 · COL23A1 · COL25A1
other: type VI ( COL6A1, COL6A2, COL6A3, COL6A5) · type VII ( COL7A1) · type VIII ( COL8A1, COL8A2) · type X ( COL10A1) · type XI ( COL11A1, COL11A2) · COL27A1 · COL28A1
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