Search on: ADRENOLEUKODYSTROPHY 
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Descriptor English:   Adrenoleukodystrophy 
Descriptor Spanish:   Adrenoleucodistrofia 
Descriptor Portuguese:   Adrenoleucodistrofia 
Synonyms English:   ALD (Adrenoleukodystrophy)
Addison Disease and Cerebral Sclerosis
Bronze Schilder Disease
Melanodermic Leukodystrophy
Siemerling-Creutzfeldt Disease
X-ALD
X-ALD (X-Linked Adrenoleukodystrophy)
Adrenoleukodystrophy, X-Linked
Leukodystrophies, Melanodermic
Leukodystrophy, Melanodermic
Schilder Addison Complex
Siemerling Creutzfeldt Disease
X ALD
X ALD (X Linked Adrenoleukodystrophy)
X Linked Adrenoleukodystrophy
Adrenomyeloneuropathy
Schilder-Addison Complex
X-Linked Adrenoleukodystrophy  
Tree Number:   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
Definition English:   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). 
Indexing Annotation English:   do not confuse with ADRENOLEUKODYSTROPHY, NEONATAL see PEROXISOMAL DISORDERS
See Related English:   ATP-Binding Cassette Transporters
 
History Note English:   1991(1983) 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
DG diagnostic imaging DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RT radiotherapy
RH rehabilitation SU surgery
TH therapy UR urine
VE veterinary VI virology
Record Number:   328 
Unique Identifier:   D000326 

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