Literature summary for 2.4.1.133 extracted from

Seidler, D.G.; Faiyaz-Ul-Haque, M.; Hansen, U.; Yip, G.W.; Zaidi, S.H.; Teebi, A.S.; Kiesel, L.; Goette, M.
Defective glycosylation of decorin and biglycan, altered collagen structure, and abnormal phenotype of the skin fibroblasts of an Ehlers-Danlos syndrome patient carrying the novel Arg270Cys substitution in galactosyltransferase I (beta4GalT-7) (2006), J. Mol. Med., 84, 583-594.

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Protein Variants

Protein Variants	Comment	Organism
R270C	compared to control fibroblasts, galactosyltransferase activity in beta4GalT-7Arg270Cys cells is approximately three times reduced over a temperature range of 25-41°C. The reduced beta4GalT-7 activity results in abnormal extracellular matrix structure due to altered decorin and biglycan glycosylation and defective collagen	Homo sapiens

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
skin fibroblast	-	Homo sapiens	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
UDP-galactose + p-nitrophenyl-beta-D-xyloside	-	Homo sapiens	UDP + Galbeta(1-4)Xylbeta-p-nitrophenyl	-	?

Synonyms

Synonyms	Comment	Organism
beta4GalT-7	-	Homo sapiens
galactosyltransferase I	-	Homo sapiens

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Release 2023.1 (January 2023)