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Literature summary for 3.2.1.46 extracted from

  • Lee, W.C.; Tsoi, Y.K.; Troendle, F.J.; DeLucia, M.W.; Ahmed, Z.; Dicky, C.A.; Dickson, D.W.; Eckman, C.B.
    Single-dose intracerebroventricular administration of galactocerebrosidase improves survival in a mouse model of globoid cell leukodystrophy (2007), FASEB J., 21, 2520-2527.
    View publication on PubMed

Application

Application Comment Organism
medicine globoid cell leukodystrophy, GLD or Krabbe disease, is caused by loss-of-fuction mutations in the GALC gene, injection of GALC improves the survival of the mouse model of GLD Mus musculus

Cloned(Commentary)

Cloned (Comment) Organism
-
Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Mus musculus 5764
-

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Mus musculus
-
twitcher mouse
-

Purification (Commentary)

Purification (Comment) Organism
-
Mus musculus

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.000008
-
GALC enzyme activity in the posterior part of the brain of single intracerebroventricular-GALC injected mice Mus musculus
0.0000087
-
GALC enzyme activity in the anterior part of the brain of single intracerebroventricular-GALC injected mice Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
D-galactosyl-N-acylsphingosine + H2O
-
Mus musculus D-galactose + N-acylsphingosine
-
?
psychosine + H2O galactosylsphingosine Mus musculus D-galactose + sphingosine
-
?

Synonyms

Synonyms Comment Organism
galactocerebrosidase
-
Mus musculus
Galactosylceramidase
-
Mus musculus
GALC
-
Mus musculus

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
GALC substrate cleavage assay Mus musculus