{"symbol":"PLOD3","synonyms":["LH3"],"name":"procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3","description":"The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VIB have deficiencies in lysyl hydroxylase activity. [provided by RefSeq, Jul 2008]","ncbiEntrezGeneId":8985,"ncbiEntrezGeneUrl":"http://www.ncbi.nlm.nih.gov/gene/8985","proteins":[{"symbol":"PLOD3_HUMAN","href":"/api/1.0/protein/PLOD3_HUMAN"}],"hgncRootFamilies":[]}