Search on: NEUROHEPATIC DEGENERATION 
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Descriptor English:   Hepatolenticular Degeneration 
Descriptor Spanish:   Degeneración Hepatolenticular 
Descriptor Portuguese:   Degeneração Hepatolenticular 
Synonyms English:   Copper Storage Disease
Hepatic Form of Wilson Disease
Hepato-Neurologic Wilson Disease
Hepatocerebral Degeneration
Hepatolenticular Degeneration Syndrome
Kinnier-Wilson Disease
Progressive Lenticular Degeneration
Westphal-Strumpell Syndrome
Wilson Disease, Hepatic Form
Wilson's Disease
Cerebral Pseudoscleroses
Degeneration, Hepatocerebral
Degeneration, Hepatolenticular
Degeneration, Neurohepatic
Degeneration, Progressive Lenticular
Degenerations, Hepatocerebral
Degenerations, Neurohepatic
Diseases, Hepato-Neurologic Wilson
Diseases, Kinnier-Wilson
Hepato Neurologic Wilson Disease
Hepato-Neurologic Wilson Diseases
Hepatocerebral Degenerations
Kinnier Wilson Disease
Kinnier-Wilson Diseases
Lenticular Degeneration, Progressive
Neurohepatic Degenerations
Pseudoscleroses, Cerebral
Pseudosclerosis, Cerebral
Westphal Strumpell Syndrome
Westphal-Strumpell Syndromes
Wilson Disease, Hepato-Neurologic
Wilson Diseases, Hepato-Neurologic
Wilsons Disease
Wilson Disease
Pseudosclerosis
Cerebral Pseudosclerosis
Neurohepatic Degeneration  
Tree Number:   C06.552.413
C10.228.140.079.493
C10.228.140.163.100.360
C10.228.662.400
C10.574.500.487
C16.320.400.361
C16.320.565.189.360
C16.320.565.618.403
C18.452.132.100.360
C18.452.648.189.360
C18.452.648.618.403
Definition English:   A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years. 
Indexing Annotation English:   lenticular refers to the lenticular nucleus in the brain
History Note English:   1964(1963) 
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:   6684 
Unique Identifier:   D006527 

Occurrence in VHL: 		 

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