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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
2000
Highly Cited
2000
Human Cu/Zn Superoxide Dismutase (SOD1) Overexpression in Mice Causes Mitochondrial Vacuolization, Axonal Degeneration, and Premature Motoneuron Death and Accelerates Motoneuron Disease in Mice…
Cytosolic Cu/Zn superoxide dismutase (SOD1) is a ubiquitous small cytosolic metalloenzyme that catalyzes the conversion of… 
Highly Cited
2000
Highly Cited
2000
Evidence That Wallerian Degeneration and Localized Axon Degeneration Induced by Local Neurotrophin Deprivation Do Not Involve Caspases
The selective degeneration of an axon, without the death of the parent neuron, can occur in response to injury, in a variety of… 
Highly Cited
1997
Highly Cited
1997
N-terminal Domains of Human Copper-transporting Adenosine Triphosphatases (the Wilson’s and Menkes Disease Proteins) Bind Copper Selectively in Vivo and in Vitro with Stoichiometry of One Copper Per…
N-terminal domains of the Wilson’s and Menkes disease proteins (N-WND and N-MNK) were overexpressed in a soluble form in… 
Highly Cited
1996
Highly Cited
1996
Chronic 3-Nitropropionic Acid Treatment in Baboons Replicates the Cognitive and Motor Deficits of Huntington’s Disease
We showed recently that chronic administration of the mitochondrial inhibitor 3-nitropropionic acid (3NP) in primates produces… 
Review
1995
Review
1995
Cellular copper transport.
Cellular copper transport processes are required by all organisms for correct utilization in cell biochemical processes and… 
Highly Cited
1993
Highly Cited
1993
High spontaneous intrachromosomal recombination rates in ataxia-telangiectasia.
Ataxia-telangiectasia (A-T) is an inherited human disease associated with neurologic degeneration, immune dysfunction, and high… 
Highly Cited
1988
Highly Cited
1988
Trimethyltin-induced neuronal damage in the rat brain: Comparative studies using silver degeneration stains, immunocytochemistry and immunoassay for neuronotypic and gliotypic proteins
Review
1978
Review
1978
Bone and joint changes in lower limb amputees.
A clinical and radiological survey of bone and joint changes in 42 lower limb amputees is reported. There was a significant… 
Highly Cited
1972
Highly Cited
1972
Projection of the cochlear nuclei on the inferior colliculus in the cat
  • K. Osen
  • The Journal of comparative neurology
  • 1972
  • Corpus ID: 24230213
The present study was carried out in order to define the topographical organization and the cells of origin of the second order… 
Highly Cited
1972
Highly Cited
1972
Thalamic relationships of the medial cortex in the rat.
Thalamic projections to the medial cortex (cingulate cortex and presubiculum) were studied by the use of anterograde degeneration… 
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