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procollagen-lysine,2-oxoglutarate 5-dioxygenase 3; lysyl hydroxylase 3; LH3 (PLOD3)
Function:
- forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens
- these hydroxylysines serve as sites of attachment for carbohydrate units & are essential for the stability of the intermolecular collagen cross-links
L-lysine-[procollagen] + 2-oxoglutarate + O2 = (2S,5R)-5-hydroxy-L-lysine-[procollagen] + succinate + CO2
Cofactor: iron, ascorbate
Structure:
- homodimer
- contains 1 Fe2OG dioxygenase domain
Compartment:
- rough endoplasmic reticulum membrane
- peripheral membrane; lumenal side
Pathology:
- defects in PLOD3 are the cause of lysyl hydroxylase 3 deficiency
Related
PLOD3 gene mutation
General
dioxygenase
glycoprotein
hydroxylase; monooxygenase
metalloprotein
Properties
SIZE: entity length = 738 aa
MW = 85 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: signal sequence {1-24}
N-glycosylation site {N63}
N-glycosylation site {N548}
Fe2OG dioxygenase {647-738}
MOTIF: Iron [Fe]-binding site
SITE: 667-667
Iron [Fe]-binding site
SITE: 669-669
Iron [Fe]-binding site
SITE: 719-719
arginine residue {R729}
Database Correlations
OMIM correlations
MORBIDMAP 603066
UniProt O60568
Pfam PF03171
Entrez Gene 8985
KEGG correlations
ENZYME 1.14.11.4
References
UniProt :accession O60568