"Adrenoleukodystrophy" 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 X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS).
Descriptor ID |
D000326
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MeSH Number(s) |
C10.228.140.163.100.084 C10.228.140.163.100.362.250 C10.228.140.695.625.250 C10.314.400.250 C10.597.606.643.455.124 C16.320.322.500.124 C16.320.400.525.124 C16.320.565.189.084 C16.320.565.189.362.250 C16.320.565.663.100 C18.452.132.100.084 C18.452.132.100.362.250 C18.452.648.189.084 C18.452.648.189.362.250 C18.452.648.663.100 C19.053.500.270
|
Concept/Terms |
Adrenoleukodystrophy- Adrenoleukodystrophy
- Melanodermic Leukodystrophy
- Leukodystrophies, Melanodermic
- Leukodystrophy, Melanodermic
- Siemerling-Creutzfeldt Disease
- Siemerling Creutzfeldt Disease
- Addison Disease and Cerebral Sclerosis
- X-ALD
- X ALD
- Bronze Schilder Disease
- X-Linked Adrenoleukodystrophy
- Adrenoleukodystrophy, X-Linked
- X Linked Adrenoleukodystrophy
- ALD (Adrenoleukodystrophy)
- X-ALD (X-Linked Adrenoleukodystrophy)
- X ALD (X Linked Adrenoleukodystrophy)
- Schilder-Addison Complex
- Schilder Addison Complex
|
Below are MeSH descriptors whose meaning is more general than "Adrenoleukodystrophy".
- 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]
- Adrenoleukodystrophy [C10.228.140.163.100.084]
- Hereditary Central Nervous System Demyelinating Diseases [C10.228.140.163.100.362]
- Adrenoleukodystrophy [C10.228.140.163.100.362.250]
- Leukoencephalopathies [C10.228.140.695]
- Hereditary Central Nervous System Demyelinating Diseases [C10.228.140.695.625]
- Adrenoleukodystrophy [C10.228.140.695.625.250]
- Demyelinating Diseases [C10.314]
- Hereditary Central Nervous System Demyelinating Diseases [C10.314.400]
- Adrenoleukodystrophy [C10.314.400.250]
- Neurologic Manifestations [C10.597]
- Neurobehavioral Manifestations [C10.597.606]
- Intellectual Disability [C10.597.606.643]
- Mental Retardation, X-Linked [C10.597.606.643.455]
- Adrenoleukodystrophy [C10.597.606.643.455.124]
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
- Genetic Diseases, X-Linked [C16.320.322]
- Mental Retardation, X-Linked [C16.320.322.500]
- Adrenoleukodystrophy [C16.320.322.500.124]
- Heredodegenerative Disorders, Nervous System [C16.320.400]
- Mental Retardation, X-Linked [C16.320.400.525]
- Adrenoleukodystrophy [C16.320.400.525.124]
- Metabolism, Inborn Errors [C16.320.565]
- Brain Diseases, Metabolic, Inborn [C16.320.565.189]
- Adrenoleukodystrophy [C16.320.565.189.084]
- Hereditary Central Nervous System Demyelinating Diseases [C16.320.565.189.362]
- Adrenoleukodystrophy [C16.320.565.189.362.250]
- Peroxisomal Disorders [C16.320.565.663]
- Adrenoleukodystrophy [C16.320.565.663.100]
- Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
- Brain Diseases, Metabolic [C18.452.132]
- Brain Diseases, Metabolic, Inborn [C18.452.132.100]
- Adrenoleukodystrophy [C18.452.132.100.084]
- Hereditary Central Nervous System Demyelinating Diseases [C18.452.132.100.362]
- Adrenoleukodystrophy [C18.452.132.100.362.250]
- Metabolism, Inborn Errors [C18.452.648]
- Brain Diseases, Metabolic, Inborn [C18.452.648.189]
- Adrenoleukodystrophy [C18.452.648.189.084]
- Hereditary Central Nervous System Demyelinating Diseases [C18.452.648.189.362]
- Adrenoleukodystrophy [C18.452.648.189.362.250]
- Peroxisomal Disorders [C18.452.648.663]
- Adrenoleukodystrophy [C18.452.648.663.100]
- Endocrine System Diseases [C19]
- Adrenal Gland Diseases [C19.053]
- Adrenal Insufficiency [C19.053.500]
- Adrenoleukodystrophy [C19.053.500.270]
Below are MeSH descriptors whose meaning is more specific than "Adrenoleukodystrophy".
This graph shows the total number of publications written about "Adrenoleukodystrophy" by people in this website by year, and whether "Adrenoleukodystrophy" was a major or minor topic of these publications.
To see the data from this visualization as text,
click here.
Year | Major Topic | Minor Topic | Total |
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1996 | 1 | 1 | 2 |
1998 | 4 | 1 | 5 |
2000 | 3 | 0 | 3 |
2002 | 1 | 0 | 1 |
2003 | 1 | 0 | 1 |
2006 | 1 | 0 | 1 |
2008 | 2 | 0 | 2 |
2009 | 1 | 0 | 1 |
2010 | 2 | 0 | 2 |
2011 | 1 | 0 | 1 |
2013 | 1 | 0 | 1 |
2015 | 2 | 0 | 2 |
2016 | 1 | 0 | 1 |
2021 | 0 | 1 | 1 |
To return to the timeline,
click here.
Below are the most recent publications written about "Adrenoleukodystrophy" by people in Profiles.
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Discovery and Optimization of Pyrazole Amides as Inhibitors of ELOVL1. J Med Chem. 2021 12 23; 64(24):17753-17776.
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MicroRNA Profiling Identifies miR-196a as Differentially Expressed in Childhood Adrenoleukodystrophy and Adult Adrenomyeloneuropathy. Mol Neurobiol. 2017 03; 54(2):1392-1403.
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Functional Characterization of IPSC-Derived Brain Cells as a Model for X-Linked Adrenoleukodystrophy. PLoS One. 2015; 10(11):e0143238.
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ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy. J Neurochem. 2015 May; 133(3):380-96.
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Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes. Biochim Biophys Acta. 2013 Apr; 1831(4):747-58.
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HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes. J Lipid Res. 2011 Nov; 52(11):2056-69.
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Pathomechanisms underlying X-adrenoleukodystrophy: a three-hit hypothesis. Brain Pathol. 2010 Jul; 20(4):838-44.
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Very long-chain fatty acid accumulation causes lipotoxic response via 5-lipoxygenase in cerebral adrenoleukodystrophy. J Lipid Res. 2010 Jul; 51(7):1685-95.
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Oxidative imbalance in nonstimulated X-adrenoleukodystrophy-derived lymphoblasts. Dev Neurosci. 2008; 30(6):410-8.
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Silencing of Abcd1 and Abcd2 genes sensitizes astrocytes for inflammation: implication for X-adrenoleukodystrophy. J Lipid Res. 2009 Jan; 50(1):135-47.