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Beta-1,4-galactosyltransferase 7 (B4GALT7) is a member of the β-1,4-galactosyltransferase enzyme family responsible for the biosynthesis of the tetrasaccharide linkage region in proteoglycans, particularly in skin fibroblasts. As a type II membrane-bound glycoprotein localized to the cis-Golgi, B4GALT7 transfers galactose from UDP-galactose to specific substrates, catalyzing the critical first galactose addition in proteoglycan carbohydrate-protein linkages. The enzyme is required for the formation of structural proteoglycans in the extracellular matrix, and deficiency leads to multisystem connective tissue disorders including variants of Ehlers-Danlos syndrome and Larsen syndrome. B4GALT7 is essential in the genetics of glycosylation and extracellular matrix structure.
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