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

More general terms

• hydroxylase; monooxygenase
• dioxygenase
• glycoprotein
• metalloprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=8985
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:8985
• OMIM: https://mirror.omim.org/entry/603066
• OMIM: https://mirror.omim.org/entry/612394
• UniProt: http://www.uniprot.org/uniprot/O60568.html