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Literature summary for 2.4.1.133 extracted from

  • Goette, M.; Kresse, H.
    Defective glycosaminoglycan substitution of decorin in a patient with progeroid syndrome is a direct consequence of two point mutations in the galactosyltransferase I (beta4GalT-7) gene (2005), Biochem. Genet., 43, 65-77.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
expression in galactosyltransferase-deficient CHO618 cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information decorin is less efficiently substituted with glycosaminoglycan chains upon expression of beta4GalT-7(186D) compared to beta4GalT-7-expressing cells. Cells expressing beta4GalT-7206P do not synthesize the proteoglycan form of decorin Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
UDP-galactose + O-beta-D-xylosylprotein Homo sapiens in a progeroid patient carrying two point mutations in beta4galactosyltransferase I (ß4GalT-7) only 50% of the decorin core protein molecules are substituted with glycosaminoglycan chains. Tthe ß4GalT-7 mutations directly affect the molecular phenotype of decorin observed in a patient with the progeroid form of Ehlers-Danlos syndrome, which may be a major mechanistic cause for the skin and wound healing defects observed in this patient UDP + 4-beta-D-galactosyl-O-beta-D-xylosylprotein
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Organism

Organism UniProt Comment Textmining
Homo sapiens
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-
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
UDP-galactose + O-beta-D-xylosylprotein in a progeroid patient carrying two point mutations in beta4galactosyltransferase I (ß4GalT-7) only 50% of the decorin core protein molecules are substituted with glycosaminoglycan chains. Tthe ß4GalT-7 mutations directly affect the molecular phenotype of decorin observed in a patient with the progeroid form of Ehlers-Danlos syndrome, which may be a major mechanistic cause for the skin and wound healing defects observed in this patient Homo sapiens UDP + 4-beta-D-galactosyl-O-beta-D-xylosylprotein
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?

Synonyms

Synonyms Comment Organism
beta4galactosyltransferase I
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Homo sapiens
beta4GalT-7
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Homo sapiens