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Literature summary for 3.2.1.46 extracted from

  • Ben-Yoseph, Y.; Hungerford, M.; Nadler, H.L.
    The interrelations between high- and low-molecular weight forms of normal and mutant (Krabbe-disease) galactocerebrosidase (1980), Biochem. J., 189, 9-15.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.2.1.46

Application

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

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
121000
-
 gel filtration, monomer Homo sapiens
125000
-
 1 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE Homo sapiens
125000
-
 2 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE Homo sapiens
125000
-
 4 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE Homo sapiens
125000
-
 6 * 125000, active form in vivo, SDS-PAGE Homo sapiens
760000
-
 gel filtration, hexamer Homo sapiens

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
 normal enzyme
-
Homo sapiens
-
 mutant enzyme /Krabbe disease
-

Purification (Commentary)

Purification (Comment) Organism
to homogeneity, Krabbe disease Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Homo sapiens
-
liver
-
 Homo sapiens
-

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
dimer 2 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE Homo sapiens
hexamer 6 * 125000, active form in vivo, SDS-PAGE Homo sapiens
monomer 1 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE Homo sapiens
tetramer 4 * 125000, aggregation from monomer to dimer, from dimer to tetramer or hexamer, SDS-PAGE Homo sapiens