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GALACTOSYLCERAMIDASE

Known as: Galactocerebrosidase, D-Galactosyl-N-acylsphingosine galactohydrolase, Galactosidase, Galactosylceramide 
An enzyme that hydrolyzes galactose from ceramide monohexosides. Deficiency of this enzyme may cause globoid cell leukodystrophy (LEUKODYSTROPHY… 
National Institutes of Health

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11 relations
GALC geneGalactocerebrosidase, humanGloboid cell leukodystrophyIn Blood

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lactosylceramidase

Papers overview

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2015
2015
Mitigation of cerebellar neuropathy in globoid cell leukodystrophy mice by AAV-mediated gene therapy.
2006
2006
Molecular basis of globoid cell leukodystrophy in Irish setters.
2000
2000
Deletion of exons 11–17 and novel mutations of the galactocerebrosidase gene in adult- and early-onset patients with Krabbe disease
Sirs: Globoid cell leukodystrophy (GLD, or Krabbe disease) is an autosomal recessive disease that usually begins during infancy… 
1999
1999
Transduction of Cultured Oligodendrocytes from Normal and Twitcher Mice by a Retroviral Vector Containing Human Galactocerebrosidase (GALC) cDNA
Krabbe disease or globoid cell leukodystropy is a lysosomal disorder caused by a deficiency of galactocerebrosidase (GALC… 
1998
1998
Retroviral‐mediated transfer of the galactocerebrosidase gene in neural progenitor cells
GLOBOID cell leukodystrophy (GCL or Krabbe disease) is a recessive disease caused by mutations of the lysosomal enzyme… 
1991
1991
Rapid kidney changes resulting from glycosphingolipid depletion by treatment with a glucosyltransferase inhibitor.
1983
1983
Deficiency of lysosomal hydrolases in apparently healthy individuals.
The deficiency of a lysosomal hydrolase usually results in the storage of its substrate(s) leading to various clinical… 
1981
1981
Congenital muscle fiber-type disproportion in Krabbe's leukodystrophy.
Progressive spasticity, blindness, loss of skills, and neuropathy developed in a 4.5-month-old boy. When examined at 13 months… 
1981
1981
Effect of suramin on the activities of degradative enzymes of sphingolipids in rats.
Three to nine days after administration of suramin, 500 mg/kg intravenously in rats, a small amount of the drug (about 0.25… 
1977
1977
Substrate specificities of the two genetically distinct human brain β-galactosidases
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