Procollagen galactosyltransferase

procollagen galactosyltransferase
Identifiers
EC no.2.4.1.50
CAS no.9028-07-3
Databases
BRENDAenzyme data
ExPASyNiceZyme view
KEGGenzyme entry
MetaCycmetabolic pathway
Rheareactions
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
Search
PMCarticles
PubMedarticles
NCBIproteins

In enzymology, a procollagen galactosyltransferase (EC 2.4.1.50) is an enzyme that catalyzes the chemical reaction

UDP-galactose + procollagen 5-hydroxy-L-lysine UDP + procollagen 5-(D-galactosyloxy)-L-lysine

Thus, the two substrates of this enzyme are UDP-galactose and procollagen 5-hydroxy-L-lysine, whereas its two products are UDP and procollagen 5-(D-galactosyloxy)-L-lysine.

Nomenclature

  • Family: glycosyltransferases, specifically the hexosyltransferases
  • Systematic name: UDP-galactose D-galactosyltransferase
  • Other names:
    • hydroxylysine galactosyltransferase
    • collagen galactosyltransferase
    • collagen hydroxylysyl galactosyltransferase
    • UDP galactose-collagen galactosyltransferase
    • uridine diphosphogalactose-collagen galactosyltransferase
    • UDPgalactose:5-hydroxylysine-collagen galactosyltransferase
  • Pathway lysine degradation

Human genes

Human genes encoding enzymes with procollagen galactosyltransferase activity include: Human genes encoding enzymes with procollagen galactosyltransferase activity (EC 2.4.1.50) include:[1][2][3]

Structure and functions of procollagen galactosyltransferase 1

Procollagen galactosyltransferase 1 (GT251), encoded by the COLGALT1 gene, plays a crucial role in lysyl O-linked glycosylation and the maturation of collagen.[4] GT251 consists of two galactosyltransferase domains (GalT-N and GalT-C) and is stabilized in a dimeric form.[5] The GT251 dimer can further associate with LH3 (encoded by PLOD3) to form a heterotetrameric complex, known as the KOGG complex (Lysyl Hydroxylation-Galactosylation-Glucosylation complex).[5] Within this complex, three key enzymatic reactions in lysine O-linked glycosylation are coordinately catalyzed by LH3 and GT251, ensuring proper collagen modification and structural integrity.

References

  1. 1 2 "COLGALT1 – Procollagen galactosyltransferase 1". UniProt. UniProt Consortium. Retrieved 16 August 2026.
  2. 1 2 "COLGALT2 – Procollagen galactosyltransferase 2". UniProt. UniProt Consortium. Retrieved 16 August 2026.
  3. 1 2 "PLOD3 – Multifunctional procollagen lysine hydroxylase and glycosyltransferase LH3". UniProt. UniProt Consortium. Retrieved 16 August 2026.
  4. ↑ Schegg B, Hülsmeier AJ, Rutschmann C, Maag C, Hennet T (February 2009). "Core glycosylation of collagen is initiated by two beta(1-O)galactosyltransferases". Molecular and Cellular Biology. 29 (4): 943–952. doi:10.1128/MCB.02085-07. PMC 2643808. PMID 19075007.
  5. 1 2 Peng J, Li W, Yao D, Xia Y, Wang Q, Cai Y, et al. (March 2025). "The structural basis for the human procollagen lysine hydroxylation and dual-glycosylation". Nature Communications. 16 (1) 2436. Bibcode:2025NatCo..16.2436P. doi:10.1038/s41467-025-57768-9. PMC 11897130. PMID 40069201.

Further reading