Lysyl hydroxylase

procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase 1
Identifiers
Symbol	PLOD1
Alt. symbols	LLH, PLOD
Chr. 1 p36.3-36.2
Search for Structures Swiss-model Domains InterPro

procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2
Identifiers
Symbol	PLOD2
Chr. 3 q24
Search for Structures Swiss-model Domains InterPro

Lysyl hydroxylases (or procollagen-lysine 5-dioxygenases) are

. There are three lysyl hydroxylases (LH1-3) encoded in the human genome, namely: PLOD1, PLOD2 and PLOD3. From PLOD2 two splice variant can be expressed (LH2a and LH2b), where LH2b differs from LH2a by incorporating the small exon 13A. LH1 and LH3 hydroxylate lysyl residues in the collagen triple helix, whereas LH2b hydroxylates lysyl residues in the telopeptides of collagen. In addition to its hydroxylation activity, LH3 has glycosylation activity that produces either monosaccharide (Gal) or disaccharide (Glc-Gal) attached to collagen hydroxylysines.

Collagen lysyl hydroxylation is the first step in collagen pyridinoline cross-linking, that is necessary for the stabilization of collagen.

Pathology

Mutations in the PLOD1 gene have been linked to

Mutations in the PLOD2 gene have been linked to Bruck syndrome in humans.

A deficiency in its cofactor vitamin C is associated with scurvy.

References

External links

• Lysyl+Hydroxylase at the U.S. National Library of Medicine Medical Subject Headings (MeSH)

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