Globoid Cell Leukodystrophy (Krabbe Disease)

An autosomal recessive metabolic disorder caused by a deficiency of GALACTOSYLCERAMIDASE leading to intralysosomal accumulation of galactolipids such as GALACTOSYLCERAMIDES and PSYCHOSINE. It is characterized by demyelination associated with large multinucleated globoid cells, predominantly involving the white matter of the central nervous system. The loss of MYELIN disrupts normal conduction of nerve impulses.
Also Known As:
Krabbe Disease; Leukodystrophy, Globoid; Krabbe Leukodystrophy; Krabbe's Disease; Leukodystrophy, Krabbe; Diffuse Globoid Body Sclerosis; Galactosylceramidase Deficiency Disease; Leukodystrophy, Globoid Cell; Classic Globoid Cell Leukodystrophy; Early-Onset Globoid Cell Leukodystrophy; Galactosylceramide-beta-Galactosidase Deficiency Disease; Globoid Body Sclerosis, Diffuse; Globoid Cell Leukoencephalopathy; Globoid Leukodystrophy; Infantile Globoid Cell Leukodystrophy; Krabbe's Leukodystrophy; Late-Onset Globoid Cell Leukodystrophy; Leukodystrophy, Globoid Cell, Classic; Leukodystrophy, Globoid Cell, Early-Onset; Leukodystrophy, Globoid Cell, Infantile; Leukodystrophy, Globoid Cell, Late-Onset; Cell Leukodystrophies, Globoid; Cell Leukodystrophy, Globoid; Cell Leukoencephalopathies, Globoid; Cell Leukoencephalopathy, Globoid; Deficiency Disease, Galactosylceramidase; Deficiency Disease, Galactosylceramide-beta-Galactosidase; Deficiency Diseases, Galactosylceramidase; Deficiency Diseases, Galactosylceramide-beta-Galactosidase; Disease, Galactosylceramidase Deficiency; Disease, Galactosylceramide-beta-Galactosidase Deficiency; Diseases, Galactosylceramidase Deficiency; Diseases, Galactosylceramide-beta-Galactosidase Deficiency; Early Onset Globoid Cell Leukodystrophy; Galactosylceramidase Deficiency Diseases; Galactosylceramide beta Galactosidase Deficiency Disease; Galactosylceramide-beta-Galactosidase Deficiency Diseases; Globoid Cell Leukodystrophies; Globoid Cell Leukoencephalopathies; Globoid Leukodystrophies; Krabbes Disease; Krabbes Leukodystrophy; Late Onset Globoid Cell Leukodystrophy; Leukodystrophies, Globoid; Leukodystrophies, Globoid Cell; Leukodystrophy, Krabbe's; Leukoencephalopathies, Globoid Cell; Leukoencephalopathy, Globoid Cell
Networked: 416 relevant articles (17 outcomes, 26 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Globoid Cell Leukodystrophy (Krabbe Disease)
2. Demyelinating Diseases (Demyelinating Disease)
3. Metachromatic Leukodystrophy (Sulfatide Lipidosis)
4. Gaucher Disease (Gaucher's Disease)
5. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)


1. Wenger, David A: 17 articles (11/2015 - 04/2004)
2. Sands, Mark S: 12 articles (04/2015 - 07/2004)
3. Bongarzone, Ernesto R: 12 articles (01/2015 - 02/2007)
4. Rafi, Mohammad A: 9 articles (11/2015 - 05/2005)
5. Singh, Inderjit: 9 articles (01/2015 - 05/2002)
6. Bunnell, Bruce A: 9 articles (02/2014 - 06/2007)
7. Martino, Sabata: 8 articles (06/2015 - 03/2009)
8. Lopez-Rosas, Aurora: 8 articles (01/2015 - 12/2007)
9. Singh, Avtar K: 8 articles (01/2015 - 05/2002)
10. Luzi, Paola: 7 articles (11/2015 - 05/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Globoid Cell Leukodystrophy:
1. GalactosylceramidaseIBA
2. Psychosine (Galactosylsphingosine)IBA
3. beta-Galactosidase (Lactaid)IBA
4. galactocerebrosideIBA
5. Galactocerebrosidase deficiencyIBA
6. Galactose (Galactopyranose)FDA LinkGeneric
7. CerebrosidesIBA
8. IsoflavonesIBA
9. Dimethyl Sulfoxide (DMSO)FDA LinkGeneric
10. AntioxidantsIBA

Therapies and Procedures

1. Bone Marrow Transplantation (Transplantation, Bone Marrow)
2. Hematopoietic Stem Cell Transplantation
3. Cell Transplantation
4. Transplants (Transplant)
5. Enzyme Replacement Therapy