Target intelligence / Profile preview

Collagen beta(1-O)galactosyltransferase 1 (COLGALT1)

Target
COLGALT1
Molecular classification
Enzyme, Glycosyltransferase
01

Overview

Collagen beta(1-O)galactosyltransferase 1 (COLGALT1) is an enzyme that transfers beta-galactose to hydroxylysine residues in collagen, facilitating the formation of the collagen triple helix structure. It plays a crucial role in extracellular matrix organization and collagen stability. Dysfunctions in COLGALT1 have been associated with various collagen-related disorders, including brain small vessel disease and familial porencephaly. The enzyme acts within the endoplasmic reticulum and is involved in the biosynthesis of collagen types I and IV. COLGALT1 forms part of the procollagen lysine hydroxylation and glycosylation complex (KOGG complex) with other enzymes like LH3, contributing to the proper assembly and maturation of collagens.

Other names
Procollagen galactosyltransferase 1GLT25D1ColGalT 1FLJ22329Glycosyltransferase 25 family member 1Hydroxylysine galactosyltransferase 1BSVD3
02

Biological functions

Collagen glycosylationCollagen triple helix formationExtracellular matrix organization
03

Disease associations

Brain Small Vessel DiseaseFamilial PorencephalyCollagen-related disorders

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