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.

Pathology

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

External links

v • d • e
Oxidoreductases: dioxygenases, including steroid hydroxylases (EC 1.14)
1.14.11 - 2-oxoglutarate
Prolyl hydroxylase - Lysyl hydroxylase
1.14.13 - NADH or NADPH
1.14.14 - reduced flavin or flavoprotein
1.14.15 - reduced iron-sulfur protein
1.14.16 - reduced pteridine
1.14.17 - reduced ascorbate
1.14.18-19 - other
1.14.99 - miscellaneous
v • d • e
Metabolism: amino acid metabolism - synthesis and catabolism enzymes (essential in CAPS)
K→acetyl-CoA
(see below)
G→pyruvate
G→glutamate
G→propionyl-CoA
→succinyl-CoA→TCA
G→fumarate
G→oxaloacetate
Other
Prolyl hydroxylase - Lysyl hydroxylase
 This oxidoreductase article is a stub. You can help Wikipedia by expanding it.

Wikipedia content modification information:

  • This page was last modified on 11 October 2008, at 07:17.

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