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Hepatolenticular Degeneration

Known as: Wilson's Disease, Hepatocerebral Degeneration, Pseudoscleroses, Cerebral 
A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects… 
National Institutes of Health

Related topics

Related topics

41 relations
ATP7B geneAminoaciduriaAnemia, HemolyticAutosomal recessive inheritance

Broader (2)

Disorders of copper metabolismLiver diseases

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2006
Highly Cited
2006
The Rice Tapetum Degeneration Retardation Gene Is Required for Tapetum Degradation and Anther Development[W]
In flowering plants, tapetum degeneration is proposed to be triggered by a programmed cell death (PCD) process during late stages… 
Highly Cited
1996
Highly Cited
1996
Preserved neuron number in the hippocampus of aged rats with spatial learning deficits.
Hippocampal neuron loss is widely viewed as a hallmark of normal aging. Moreover, neuronal degeneration is thought to contribute… 
Highly Cited
1995
Highly Cited
1995
Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models
Huntington disease (HD) is an inherited neurodegenerative disorder characterized by selective death of striatal medium spiny… 
Highly Cited
1993
Highly Cited
1993
Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
Review
1992
Review
1992
Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses?
The etiology of nerve cell death in neuronal degenerative diseases is unknown, but it has been hypothesized that excitotoxic… 
Highly Cited
1984
Highly Cited
1984
A study of computer-assisted tomography. I. The incidence of positive CAT scans in an asymptomatic group of patients.
In order to study the type and number of CAT scan abnormalities of the lumbar spine that occur in asymptomatic people, 52 studies… 
Highly Cited
1978
Highly Cited
1978
Anthracycline cardiomyopathy monitored by morphologic changes.
Seventy-six endomyocardial biopsies obtained from 60 patients receiving adriamycin (ADM) and other anthracycline analogs were… 
Highly Cited
1975
Highly Cited
1975
The estimation of red cell superoxide dismutase activity.
A method is described for the estimation of red cell superoxide dismutase (erythrocuprein) and a normal range of activity… 
Highly Cited
1960
Highly Cited
1960
Ascending axon degeneration following anterolateral cordotomy. An experimental study in the monkey.
In the same year in which Gowers wrote the prophetic statement quoted above, Bechterew on the basis of mylogenetic studies… 
Highly Cited
1956
Highly Cited
1956
Penicillamine, a new oral therapy for Wilson's disease.
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