beta-1,4-galactosyltransferase 7; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 7; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 7 (B4GALT7)

Function

• role in glycosylation
• biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts
• protein modification; protein glycosylation

UDP-galactose + O-beta-D-xylosylprotein =>

UDP + 4-beta-D-galactosyl-O-beta-D-xylosylprotein

Cofactor: Mn+2

Structure

belongs to the glycosyltransferase 7 family

Compartment

• Golgi stack membrane
• cis cisternae of Golgi stack

Expression

• expressed in heart, pancreas & liver > placenta & kidney > brain, skeletal muscle & lung

Pathology

• defects in B4GALT7 are the cause of progeroid Ehlers-Danlos syndrome

More general terms

• beta-1,4-galactosyltransferase

Database

• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1128
• OMIM: https://mirror.omim.org/entry/130070
• OMIM: https://mirror.omim.org/entry/604327
• UniProt: http://www.uniprot.org/uniprot/Q9UBV7.html