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Literature summary for 3.2.1.46 extracted from

  • Luddi, A.; Strazza, M.; Carbone, M.; Moretti, E.; Costantino-Ceccarini, E.
    Galactosylceramidase deficiency causes sperm abnormalities in the mouse model of globoid cell leukodystrophy (2005), Exp. Cell Res., 304, 59-68.
    View publication on PubMed

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-galactosylceramide + H2O Mus musculus
-
D-galactose + ceramide
-
?
D-galactosylsphingoside + H2O Mus musculus i.e. psychosine D-galactose + sphingosine
-
?
additional information Mus musculus the enzyme is required for normal sperm maturation and function, enzyme deficiency leads to degeneration of oligodendrocytes, severe demyelination, and causes sperm abnormalities in the mouse model of human globoid cell leukodystrophy or Krabbe disease, mutant twitcher mice show reduced size of testis and sperm acrosomal membrane which is redundant, detached from the nucleus and folded over ?
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
spermatozoon
-
Mus musculus
-
testis
-
Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
D-galactosyl-alkyl-acyl-glycerol + H2O a precursor of the seminolipid Mus musculus ?
-
?
D-galactosylceramide + H2O
-
Mus musculus D-galactose + ceramide
-
?
D-galactosylsphingoside + H2O i.e. psychosine Mus musculus D-galactose + sphingosine
-
?
additional information the enzyme is required for normal sperm maturation and function, enzyme deficiency leads to degeneration of oligodendrocytes, severe demyelination, and causes sperm abnormalities in the mouse model of human globoid cell leukodystrophy or Krabbe disease, mutant twitcher mice show reduced size of testis and sperm acrosomal membrane which is redundant, detached from the nucleus and folded over Mus musculus ?
-
?

Synonyms

Synonyms Comment Organism
GALC
-
Mus musculus