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
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 that catalyzes the hydroxylation of lysine to hydroxylysine.[1][2] 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.

Pathology[edit]

A deficiency in its cofactor, Vitamin C, is associated with Scurvy.

References[edit]

  1. ^ Hausmann, E. (1967). "Cofactor requirements for the enzymatic hydroxylation of lysine in a polypeptide precursor of collagen". Biochim. Biophys. Acta 133 (3): 591–598. doi:10.1016/0005-2795(67)90566-1. PMID 6033801. 
  2. ^ Rhoads, R.E. and Udenfriend, S. (1968). "Decarboxylation of α-ketoglutarate coupled to collagen proline hydroxylase". Proc. Natl. Acad. Sci. USA 60 (4): 1473–1478. doi:10.1073/pnas.60.4.1473. PMC 224943. PMID 5244754. 

External links[edit]