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Hereditary Neurodegenerative Disorder

An inherited disorder characterized by progressive degeneration and atrophy of the nervous system.
National Institutes of Health

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Central Nervous SystemNervous system structure

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2019
2019
Subthalamic and pallidal oscillatory activity in patients with Neurodegeneration with Brain Iron Accumulation type I (NBIA-I)
Review
2019
Review
2019
The psychopharmacology of Huntington disease.
2013
2013
Successful deep brain stimulation surgery with intraoperative magnetic resonance imaging on a difficult neuroacanthocytosis case: case report.
BACKGROUND AND IMPORTANCE Chorea acanthocytosis is a progressive hereditary neurodegenerative disorder characterized by… 
2013
2013
Study of oxidative damage and antioxidant systems in two Huntington's disease rodent models.
Huntington's disease (HD) is a hereditary neurodegenerative disorder, characterized by motor, psychiatric, and cognitive symptoms… 
Review
2012
Review
2012
Screening of Therapeutic Strategies for Huntington's Disease in YAC128 Transgenic Mice
Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by an unstable expansion of cytosine‐adenine‐guanine… 
2006
2006
Normal sensitivity to excitotoxicity in a transgenic Huntington's disease rat
2006
2006
A neuroendocrine dysfunction, not testicular mutant ataxin-3 cleavage fragment or aggregate, causes cell death in testes of transgenic mice
A neuroendocrine dysfunction, not testicular mutant ataxin-3 cleavage fragment or aggregate, causes cell death in testes of… 
2001
2001
Mice transgenic for exon 1 of the Huntington's disease gene display reduced striatal sensitivity to neurotoxicity induced by dopamine and 6‐hydroxydopamine
Huntington's disease is an autosomal dominant hereditary neurodegenerative disorder characterized by severe striatal cell loss… 
1983
1983
Pseudo arylsulfatase-A deficiency in healthy individuals: genetic and biochemical relationship to metachromatic leukodystrophy.
Metachromatic leukodystrophy is a hereditary neurodegenerative disorder in man associated with deficient arylsulfatase-A activity… 
1983
1983
Kainic acid: insights from a neurotoxin into the pathophysiology of Huntington's disease.
Kainic acid, a conformationally restricted analog of L-glutamic acid, is a potent neuroexcitant. Consistent with the excitotoxin… 
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