An enzyme that hydrolyzes galactose from ceramide monohexosides. Deficiency of this enzyme may cause globoid cell leukodystrophy (LEUKODYSTROPHY, GLOBOID CELL). EC
Also Known As:
Galactosylceramide Galactosidase; Galactosylceramide beta-Galactosidase; Galactosylcerebroside beta-Galactosidase; Galactosidase, Galactosylceramide; beta-Galactosidase, Galactosylceramide; beta-Galactosidase, Galactosylcerebroside; Galactocerebrosidase; D-Galactosyl-N-acylsphingosine galactohydrolase
Networked: 223 relevant articles (4 outcomes, 5 trials/studies)

Relationship Network

Bio-Agent Context: Research Results


1. Wenger, David A: 14 articles (11/2015 - 04/2004)
2. Sands, Mark S: 10 articles (04/2015 - 09/2005)
3. Rafi, Mohammad A: 9 articles (11/2015 - 05/2005)
4. Martino, Sabata: 8 articles (06/2015 - 03/2009)
5. Luzi, Paola: 7 articles (11/2015 - 05/2005)
6. Gritti, Angela: 6 articles (06/2015 - 03/2009)
7. Luddi, Alice: 6 articles (03/2015 - 05/2009)
8. Costantino-Ceccarini, Elvira: 6 articles (11/2014 - 05/2009)
9. Bongarzone, Ernesto R: 6 articles (10/2014 - 12/2007)
10. Bunnell, Bruce A: 6 articles (02/2014 - 01/2008)

Related Diseases

1. Globoid Cell Leukodystrophy (Krabbe Disease)
03/01/2009 - "The determination of cellular beta-galactocerebrosidase activity is an established procedure to diagnose Krabbe disease and monitor the efficacy of gene/stem cell-based therapeutic approaches aimed at restoring defective enzymatic activity in patients or disease models. "
02/01/2008 - "To investigate the effects of Tat-PTD addition on the subcellular localization of the lysosomal enzyme galactocerebrosidase (GALC, EC and with a view towards designing improved therapeutic strategies for Krabbe disease (globoid cell leukodystrophy), mouse GALC was tagged C-terminally with the Tat-PTD. "
08/01/2012 - "To assess the utility of a telephone-based interview system in providing ongoing monitoring of the developmental and functional status of children with both positive newborn screens for Krabbe disease and low galactocerebrosidase activity on confirmatory testing, and to determine whether this approach provides improved compliance with follow-up compared with formal neuropsychological testing. "
06/01/2009 - "This study characterized the therapeutic benefits of combining hematogenous cell replacement with lentiviral-mediated gene transfer of galactosylceramidase (GALC) in Twitcher mice, a bona fide model for Krabbe disease. "
01/01/1997 - "We report here a detailed ultrastructural study of a brain biopsy along with post-mortem brain and optic nerve specimens from a case of Krabbe disease, a relatively rare leukodystrophy caused by a mutation in the gene for galactocerebrosidase (GALC) mapped to the 14q31 region of chromosome 14. "
2. Central Nervous System Diseases (CNS Diseases)
3. Demyelinating Diseases (Demyelinating Disease)
4. Neurodegenerative Diseases (Neurodegenerative Disease)
5. Lysosomal Storage Diseases (Lysosomal Storage Disease)

Related Drugs and Biologics

1. Psychosine (Galactosylsphingosine)
2. DNA (Deoxyribonucleic Acid)
3. beta-Galactosidase (Lactaid)
4. Galactocerebrosidase deficiency
5. galactocerebroside
6. Complementary DNA (cDNA)
7. Galactose (Galactopyranose)
8. Saposins
9. acid beta-galactosidase
10. Sphingolipids

Related Therapies and Procedures

1. Bone Marrow Transplantation (Transplantation, Bone Marrow)
2. Cell Transplantation
3. Transplantation (Transplant Recipients)