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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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Highly Cited
2006
Highly Cited
2006
The therapeutic potential of neural stem/progenitor cells in murine globoid cell leukodystrophy is conditioned by macrophage/microglia activation
2006
2006
Design and optimization of lentiviral vectors for transfer of GALC expression in Twitcher brain
Demyelination in globoid cell leukodystrophy (GLD) is due to a deficiency of galactocerebrosidase (GALC) activity. Up to now, in… 
Highly Cited
2001
Highly Cited
2001
Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements
Twitcher mouse is a murine model of human globoid cell leukodystrophy (Krabbe disease), which is characterized by a genetic… 
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… 
Highly Cited
1996
Highly Cited
1996
Multiple mutations in the GALC gene in a patient with adult‐onset krabbe disease
A 53‐year‐old man was diagnosed 8 years earlier with globod cell leukodystrophy (GLD, Krabbe disease) by his severe deficiency of… 
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… 
1983
1983
Abnormal accumulation of galactosylceramide in the kidney of twitcher mouse.
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… 
1977
1977
Substrate specificities of the two genetically distinct human brain β-galactosidases
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