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Literature summary extracted from

  • Ben-Yoseph, Y.; Hungerford, M.; Nadler, H.L.
    Galactosylceramide beta-galactosidase in Krabbe disease: partial purification and characterization of the mutant enzyme (1979), Arch. Biochem. Biophys., 196, 93-101.
    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

General Stability

EC Number General Stability Organism
3.2.1.46 urea, 50% loss of activity of the mutant enzyme at 1.3 M urea, 50% loss of activity of the normal enzyme at 5.6 M urea Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.2.1.46 0.02
-
 galactosylceramide normal enzyme Homo sapiens
3.2.1.46 0.165
-
 galactosylceramide mutant enzyme Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.46 750000
-
 gel filtration, SDS-PAGE, mutant and normal protein 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
-

Purification (Commentary)

EC Number Purification (Comment) Organism
3.2.1.46
-
 Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.46 liver
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.2.1.46 0.12
-
-
 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
-
 ?
3.2.1.46 galactosylceramide + H2O
-
 Homo sapiens D-galactose + N-acylceramide
-
 ?
3.2.1.46 lactosylsphingosine + H2O
-
 Homo sapiens lactose + sphingosine
-
 ?

Temperature Stability [°C]

EC Number Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
3.2.1.46 52
-
 4 min, 50% loss of activity, mutant enzyme Homo sapiens
3.2.1.46 52
-
 35 min, 50% loss of activity, normal enzyme Homo sapiens