Lysyl hydroxylase

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

Overview

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.

Pathology

A deficiency is associated with Ehlers-Danlos syndrome.

External links

• MeSH Lysyl+Hydroxylase
• l_16/12507544 at Dorland's Medical Dictionary