Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 is an enzyme that in humans is encoded by the PLOD3 gene.[5][6][7]

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.[7]

References

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000106397Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000004846Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Passoja K, Rautavuoma K, Ala-Kokko L, Kosonen T, Kivirikko KI (Sep 1998). "Cloning and characterization of a third human lysyl hydroxylase isoform". Proc Natl Acad Sci U S A. 95 (18): 10482–10486. Bibcode:1998PNAS...9510482P. doi:10.1073/pnas.95.18.10482. PMC 27920. PMID 9724729.
  6. ^ Valtavaara M, Szpirer C, Szpirer J, Myllyla R (Jun 1998). "Primary structure, tissue distribution, and chromosomal localization of a novel isoform of lysyl hydroxylase (lysyl hydroxylase 3)". J Biol Chem. 273 (21): 12881–12886. doi:10.1074/jbc.273.21.12881. PMID 9582318.
  7. ^ a b "Entrez Gene: PLOD3 procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3".

Further reading