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Adrenoleukodystrophy MeSH Descriptor Data 2021


MeSH Heading
Adrenoleukodystrophy
Tree 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
C10.597.606.360.455.124
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
Unique ID
D000326
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D000326
Annotation
do not confuse with ADRENOLEUKODYSTROPHY, NEONATAL see PEROXISOMAL DISORDERS
Scope Note
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).
Entry Term(s)
ALD (Adrenoleukodystrophy)
Addison Disease and Cerebral Sclerosis
Adrenomyeloneuropathy
Bronze Schilder Disease
Melanodermic Leukodystrophy
Schilder-Addison Complex
Siemerling-Creutzfeldt Disease
X-ALD
X-ALD (X-Linked Adrenoleukodystrophy)
X-Linked Adrenoleukodystrophy
NLM Classification #
QU 265.5.P4
Previous Indexing
Adrenal Gland Hypofunction (1968-1982)
Cerebral Sclerosis, Diffuse (1966-1982)
Demyelinating Diseases (1966-1982)
See Also
ATP-Binding Cassette Transporters
Public MeSH Note
1991; see CEREBRAL SCLEROSIS, DIFFUSE 1983-1990
History Note
1991(1983)
Date Established
1991/01/01
Date of Entry
1982/04/27
Revision Date
2018/06/30
Adrenoleukodystrophy Preferred
Adrenomyeloneuropathy Related
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