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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; GALC Deficiency; Galactocerebrosidase Deficiency; Galactosylceramide Lipidosis; Galactosylceramide beta-Galactosidase Deficiency; Galactosylceramide-beta-Galactosidase Deficiency Disease; Galactosylcerebrosidase Deficiency; Galactosylsphingosine Lipidosis; 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; Psychosine Lipidosis; Cell Leukodystrophies, Globoid; Cell Leukodystrophy, Globoid; Cell Leukoencephalopathies, Globoid; Cell Leukoencephalopathy, Globoid; Deficiencies, GALC; Deficiencies, Galactocerebrosidase; Deficiencies, Galactosylceramide beta-Galactosidase; Deficiency Disease, Galactosylceramidase; Deficiency Disease, Galactosylceramide-beta-Galactosidase; Deficiency Diseases, Galactosylceramidase; Deficiency Diseases, Galactosylceramide-beta-Galactosidase; Deficiency, GALC; Deficiency, Galactocerebrosidase; Deficiency, Galactosylceramide beta-Galactosidase; Disease, Galactosylceramidase Deficiency; Disease, Galactosylceramide-beta-Galactosidase Deficiency; Diseases, Galactosylceramidase Deficiency; Diseases, Galactosylceramide-beta-Galactosidase Deficiency; Early Onset Globoid Cell Leukodystrophy; GALC Deficiencies; Galactocerebrosidase Deficiencies; Galactosylceramidase Deficiency Diseases; Galactosylceramide beta Galactosidase Deficiency; Galactosylceramide beta Galactosidase Deficiency Disease; Galactosylceramide beta-Galactosidase Deficiencies; 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; beta-Galactosidase Deficiencies, Galactosylceramide; beta-Galactosidase Deficiency, Galactosylceramide
Networked: 677 relevant articles (29 outcomes, 53 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1. Bongarzone, Ernesto R: 32 articles (01/2022 - 02/2007)
2. Wenger, David A: 27 articles (08/2021 - 04/2004)
3. Sands, Mark S: 22 articles (01/2022 - 07/2004)
4. Escolar, Maria L: 18 articles (01/2021 - 05/2005)
5. Kurtzberg, Joanne: 15 articles (10/2022 - 05/2005)
6. Rafi, Mohammad A: 12 articles (08/2021 - 05/2005)
7. Orsini, Joseph J: 12 articles (05/2021 - 06/2009)
8. Martino, Sabata: 12 articles (01/2021 - 03/2009)
9. Givogri, Maria I: 11 articles (01/2022 - 02/2007)
10. Jiang, Xuntian: 11 articles (01/2022 - 01/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Globoid Cell Leukodystrophy:
1. Psychosine (Galactosylsphingosine)IBA
2. GalactosylceramidaseIBA
3. Secretory Phospholipases A2IBA
4. AntioxidantsIBA
5. EnzymesIBA
6. beta-Galactosidase (Lactaid)IBA
7. Intravenous Immunoglobulins (IVIG)FDA Link
8. galactocerebrosideIBA
9. HydrolasesIBA
10. Galactose (Galactopyranose)FDA LinkGeneric

Therapies and Procedures

1. Therapeutics
2. Bone Marrow Transplantation (Transplantation, Bone Marrow)
3. Hematopoietic Stem Cell Transplantation
4. Transplantation
5. Cell Transplantation