Procollagen galactosyltransferase

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 ${\displaystyle \rightleftharpoons }$ 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.

This enzyme belongs to the family of glycosyltransferases, specifically the hexosyltransferases. The systematic name of this enzyme class is UDP-galactose:procollagen-5-hydroxy-L-lysine D-galactosyltransferase. Other names in common use include hydroxylysine galactosyltransferase, collagen galactosyltransferase, collagen hydroxylysyl galactosyltransferase, UDP galactose-collagen galactosyltransferase, uridine diphosphogalactose-collagen galactosyltransferase, and UDPgalactose:5-hydroxylysine-collagen galactosyltransferase. This enzyme participates in lysine degradation.

Procollagen galactosyltransferase 1 (GT251), encoded by the COLGALT1 gene, plays a crucial role in lysyl O-linked glycosylation and the maturation of collagen.^[1] GT251 consists of two galactosyltransferase domains (GalT-N and GalT-C) and is stabilized in a dimeric form.^[2] 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).^[2] 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.

• Bosmann HB, Eylar EH (1968). "Glycoprotein biosynthesis: the biosynthesis of the hydroxylysine-galactose linkage in collagen". Biochem. Biophys. Res. Commun. 33 (2): 340–6. Bibcode:1968BBRC...33..340B. doi:10.1016/0006-291X(68)90790-0. PMID 5722225.
• Kivirikko KI; Myllyla R (1979). Hall, D.A.; Jackson, D.S. (eds.). "Collagen glycosyltransferases". International Review of Connective Tissue Research. 8. New York: Academic Press: 23–72. doi:10.1016/B978-0-12-363708-6.50008-4. ISBN 9780123637086. PMID 389860.

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