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Literature summary extracted from

  • Besley, G.T.N.
    Isoelectric focusing of galactosylceramide beta-galactosylceramide beta-galactosidase in cultured skin fibroblasts of patients with Krabbes globoid-cell leucodystrophy (1975), Biochem. Soc. Trans., 3, 241-244.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.46 medicine enzyme deficiency in globoid cell leucodystrophy or Krabbe disease Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.2.1.46 D-galactosyl-N-acylsphingosine + H2O Homo sapiens
-
?
-
?

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.46 Homo sapiens
-
mutant enzyme /Krabbe disease
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.46 skin fibroblast in culture Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.46 4-methylumbelliferyl beta-galactoside + H2O
-
Homo sapiens methylumbelliferone + beta-D-galactose
-
?
3.2.1.46 D-galactosyl-N-acylsphingosine + H2O
-
Homo sapiens D-galactose + N-acylsphingosine
-
?
3.2.1.46 D-galactosyl-N-acylsphingosine + H2O
-
Homo sapiens ?
-
?
3.2.1.46 galactocerebroside + H2O
-
Homo sapiens D-galactose + N-acylceramide
-
?