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procollagen-lysine,2-oxoglutarate 5-dioxygenase 2; lysyl hydroxylase 2; LH2 (PLOD2)
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 (putative)
- ascorbate (putative)
- homodimer (putative)
Structure: contains 1 Fe2OG dioxygenase domain
Compartment:
- rough endoplasmic reticulum membrane
- peripheral membrane; lumenal side
Alternative splicing: named isoforms=2
Expression:
- highly expressed in pancreas & muscle
- isoform 1 & isoform 2 are expressed in most cell types
- isoform 2 is specifically expressed in skin, lung, dura & aorta
Pathology:
- defects in PLOD2 are the cause of Bruck syndrome type 2
Related
PLOD2 gene mutation
General
dioxygenase
glycoprotein
membrane protein
metalloprotein
phosphoprotein
Properties
SIZE: entity length = 737 aa
MW = 85 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: signal sequence {1-25}
N-glycosylation site {N63}
N-glycosylation site {N209}
N-glycosylation site {N297}
Thr phosphorylation site {T320}
Tyr phosphorylation site {Y323}
N-glycosylation site {N365}
N-glycosylation site {N522}
Fe2OG dioxygenase {644-737}
MOTIF: Iron [Fe]-binding site
SITE: 666-666
Iron [Fe]-binding site
SITE: 668-668
N-glycosylation site {N696}
Iron [Fe]-binding site
SITE: 718-718
N-glycosylation site {N725}
arginine residue {R728}
Database Correlations
OMIM correlations
MORBIDMAP 601865
UniProt O00469
Pfam PF03171
Entrez Gene 5352
Kegg hsa:5352
ENZYME 1.14.11.4
References
- UniProt :accession O00469
- Osteogenesis imperfecta variant database
Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2 (PLOD2)
http://oi.gene.le.ac.uk/home.php?select_db=PLOD2