Leukodystrophy, Globoid Cell
"Leukodystrophy, Globoid Cell" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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.
| Descriptor ID |
D007965
|
| MeSH Number(s) |
C10.228.140.163.100.362.500 C10.228.140.163.100.435.825.590 C10.228.140.695.625.500 C10.314.400.500 C16.320.565.189.362.500 C16.320.565.189.435.825.590 C16.320.565.398.641.803.585 C16.320.565.595.554.825.590 C18.452.132.100.362.500 C18.452.132.100.435.825.590 C18.452.584.687.803.585 C18.452.648.189.362.500 C18.452.648.189.435.825.590 C18.452.648.398.641.803.585 C18.452.648.595.554.825.590
|
| Concept/Terms |
Leukodystrophy, Globoid Cell- Leukodystrophy, Globoid Cell
- Cell Leukodystrophies, Globoid
- Cell Leukodystrophy, Globoid
- Globoid Cell Leukodystrophies
- Leukodystrophies, Globoid Cell
- Galactosylceramidase Deficiency Disease
- Deficiency Disease, Galactosylceramidase
- Deficiency Diseases, Galactosylceramidase
- Disease, Galactosylceramidase Deficiency
- Diseases, Galactosylceramidase Deficiency
- Galactosylceramidase Deficiency Diseases
- Galactosylceramide beta-Galactosidase Deficiency
- Deficiencies, Galactosylceramide beta-Galactosidase
- Deficiency, Galactosylceramide beta-Galactosidase
- Galactosylceramide beta Galactosidase Deficiency
- Galactosylceramide beta-Galactosidase Deficiencies
- beta-Galactosidase Deficiencies, Galactosylceramide
- beta-Galactosidase Deficiency, Galactosylceramide
- Galactosylceramide-beta-Galactosidase Deficiency Disease
- Deficiency Disease, Galactosylceramide-beta-Galactosidase
- Deficiency Diseases, Galactosylceramide-beta-Galactosidase
- Disease, Galactosylceramide-beta-Galactosidase Deficiency
- Diseases, Galactosylceramide-beta-Galactosidase Deficiency
- Galactosylceramide beta Galactosidase Deficiency Disease
- Galactosylceramide-beta-Galactosidase Deficiency Diseases
- GALC Deficiency
- Deficiencies, GALC
- Deficiency, GALC
- GALC Deficiencies
- Globoid Body Sclerosis, Diffuse
- Globoid Cell Leukodystrophy
- Globoid Cell Leukoencephalopathy
- Cell Leukoencephalopathies, Globoid
- Cell Leukoencephalopathy, Globoid
- Globoid Cell Leukoencephalopathies
- Leukoencephalopathies, Globoid Cell
- Leukoencephalopathy, Globoid Cell
- Globoid Leukodystrophy
- Globoid Leukodystrophies
- Leukodystrophies, Globoid
- Leukodystrophy, Globoid
- Krabbe Disease
- Krabbe Leukodystrophy
- Leukodystrophy, Krabbe
- Krabbe's Disease
- Krabbes Disease
- Krabbe's Leukodystrophy
- Krabbes Leukodystrophy
- Leukodystrophy, Krabbe's
- Galactosylceramide Lipidosis
- Galactosylcerebrosidase Deficiency
- Galactosylsphingosine Lipidosis
- Psychosine Lipidosis
- Diffuse Globoid Body Sclerosis
- Galactocerebrosidase Deficiency
- Deficiencies, Galactocerebrosidase
- Deficiency, Galactocerebrosidase
- Galactocerebrosidase Deficiencies
Early-Onset Globoid Cell Leukodystrophy- Early-Onset Globoid Cell Leukodystrophy
- Early Onset Globoid Cell Leukodystrophy
- Leukodystrophy, Globoid Cell, Classic
- Classic Globoid Cell Leukodystrophy
- Leukodystrophy, Globoid Cell, Infantile
- Infantile Globoid Cell Leukodystrophy
- Leukodystrophy, Globoid Cell, Early-Onset
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Below are MeSH descriptors whose meaning is more general than "Leukodystrophy, Globoid Cell".
- Diseases [C]
- Nervous System Diseases [C10]
- Central Nervous System Diseases [C10.228]
- Brain Diseases [C10.228.140]
- Brain Diseases, Metabolic [C10.228.140.163]
- Brain Diseases, Metabolic, Inborn [C10.228.140.163.100]
- Hereditary Central Nervous System Demyelinating Diseases [C10.228.140.163.100.362]
- Leukodystrophy, Globoid Cell [C10.228.140.163.100.362.500]
- Lysosomal Storage Diseases, Nervous System [C10.228.140.163.100.435]
- Sphingolipidoses [C10.228.140.163.100.435.825]
- Leukodystrophy, Globoid Cell [C10.228.140.163.100.435.825.590]
- Leukoencephalopathies [C10.228.140.695]
- Hereditary Central Nervous System Demyelinating Diseases [C10.228.140.695.625]
- Leukodystrophy, Globoid Cell [C10.228.140.695.625.500]
- Demyelinating Diseases [C10.314]
- Hereditary Central Nervous System Demyelinating Diseases [C10.314.400]
- Leukodystrophy, Globoid Cell [C10.314.400.500]
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
- Metabolism, Inborn Errors [C16.320.565]
- Brain Diseases, Metabolic, Inborn [C16.320.565.189]
- Hereditary Central Nervous System Demyelinating Diseases [C16.320.565.189.362]
- Leukodystrophy, Globoid Cell [C16.320.565.189.362.500]
- Lysosomal Storage Diseases, Nervous System [C16.320.565.189.435]
- Sphingolipidoses [C16.320.565.189.435.825]
- Leukodystrophy, Globoid Cell [C16.320.565.189.435.825.590]
- Lipid Metabolism, Inborn Errors [C16.320.565.398]
- Lipidoses [C16.320.565.398.641]
- Sphingolipidoses [C16.320.565.398.641.803]
- Leukodystrophy, Globoid Cell [C16.320.565.398.641.803.585]
- Lysosomal Storage Diseases [C16.320.565.595]
- Lysosomal Storage Diseases, Nervous System [C16.320.565.595.554]
- Sphingolipidoses [C16.320.565.595.554.825]
- Leukodystrophy, Globoid Cell [C16.320.565.595.554.825.590]
- Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
- Brain Diseases, Metabolic [C18.452.132]
- Brain Diseases, Metabolic, Inborn [C18.452.132.100]
- Hereditary Central Nervous System Demyelinating Diseases [C18.452.132.100.362]
- Leukodystrophy, Globoid Cell [C18.452.132.100.362.500]
- Lysosomal Storage Diseases, Nervous System [C18.452.132.100.435]
- Sphingolipidoses [C18.452.132.100.435.825]
- Leukodystrophy, Globoid Cell [C18.452.132.100.435.825.590]
- Lipid Metabolism Disorders [C18.452.584]
- Lipidoses [C18.452.584.687]
- Sphingolipidoses [C18.452.584.687.803]
- Leukodystrophy, Globoid Cell [C18.452.584.687.803.585]
- Metabolism, Inborn Errors [C18.452.648]
- Brain Diseases, Metabolic, Inborn [C18.452.648.189]
- Hereditary Central Nervous System Demyelinating Diseases [C18.452.648.189.362]
- Leukodystrophy, Globoid Cell [C18.452.648.189.362.500]
- Lysosomal Storage Diseases, Nervous System [C18.452.648.189.435]
- Sphingolipidoses [C18.452.648.189.435.825]
- Leukodystrophy, Globoid Cell [C18.452.648.189.435.825.590]
- Lipid Metabolism, Inborn Errors [C18.452.648.398]
- Lipidoses [C18.452.648.398.641]
- Sphingolipidoses [C18.452.648.398.641.803]
- Leukodystrophy, Globoid Cell [C18.452.648.398.641.803.585]
- Lysosomal Storage Diseases [C18.452.648.595]
- Lysosomal Storage Diseases, Nervous System [C18.452.648.595.554]
- Sphingolipidoses [C18.452.648.595.554.825]
- Leukodystrophy, Globoid Cell [C18.452.648.595.554.825.590]
Below are MeSH descriptors whose meaning is more specific than "Leukodystrophy, Globoid Cell".
This graph shows the total number of publications written about "Leukodystrophy, Globoid Cell" by people in this website by year, and whether "Leukodystrophy, Globoid Cell" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2002 | 2 | 0 | 2 |
| 2003 | 1 | 0 | 1 |
| 2005 | 1 | 0 | 1 |
| 2006 | 2 | 0 | 2 |
| 2007 | 1 | 0 | 1 |
| 2008 | 2 | 0 | 2 |
| 2010 | 1 | 0 | 1 |
| 2013 | 1 | 0 | 1 |
| 2014 | 1 | 0 | 1 |
| 2016 | 1 | 0 | 1 |
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Below are the most recent publications written about "Leukodystrophy, Globoid Cell" by people in Profiles.
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Biochemical, cell biological, pathological, and therapeutic aspects of Krabbe's disease. J Neurosci Res. 2016 11; 94(11):990-1006.
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Effect of vitamin D3 intake on the onset of disease in a murine model of human Krabbe disease. J Neurosci Res. 2015 Jan; 93(1):28-42.
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Role of endogenous psychosine accumulation in oligodendrocyte differentiation and survival: implication for Krabbe disease. Brain Res. 2013 May 01; 1508:44-52.
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Factors that affect postnatal bone growth retardation in the twitcher murine model of Krabbe disease. Biochim Biophys Acta. 2010 Jul-Aug; 1802(7-8):601-8.
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Psychosine-induced alterations in peroxisomes of twitcher mouse liver. Arch Biochem Biophys. 2008 Sep 15; 477(2):211-8.
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The role of AMPK in psychosine mediated effects on oligodendrocytes and astrocytes: implication for Krabbe disease. J Neurochem. 2008 Jun; 105(5):1820-33.
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Autonomic denervation of lymphoid organs leads to epigenetic immune atrophy in a mouse model of Krabbe disease. J Neurosci. 2007 Dec 12; 27(50):13730-8.
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Krabbe disease: psychosine-mediated activation of phospholipase A2 in oligodendrocyte cell death. J Lipid Res. 2006 Jul; 47(7):1478-92.
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Dysfunction of peroxisomes in twitcher mice brain: a possible mechanism of psychosine-induced disease. Biochem Biophys Res Commun. 2006 Apr 28; 343(1):229-38.
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Peroxisomal participation in psychosine-mediated toxicity: implications for Krabbe's disease. J Neurosci Res. 2005 Jun 15; 80(6):845-54.