Lysyl hydroxylase

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procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase 1
Identifiers
Symbol PLOD1
Alt. Symbols LLH, PLOD
Entrez 5351
HUGO 9081
OMIM 153454
RefSeq NM_000302
UniProt Q02809
Other data
EC number 1.14.11.4
Locus Chr. 1 p36.3-36.2
procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2
Identifiers
Symbol PLOD2
Entrez 5352
HUGO 9082
OMIM 601865
RefSeq NM_000935
UniProt O00469
Other data
Locus Chr. 3 q24

Lysyl hydroxylase (or procollagen-lysine 5-dioxygenase) is an oxygenase enzyme which catalyzes the hydroxylation of lysine to hydroxylysine. This reaction is involved in the formation and stabilization of collagen.

It requires vitamin C as a cofactor.

[edit] Pathology

A deficiency is associated with Ehlers-Danlos syndrome (type 6).

[edit] External links