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Huntington Disease
Research News and Information
Definition of 'Huntington Disease'A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4) |
Monday, November 23, 2009
Lycopene modulates nitric oxide pathways against 3-nitropropionic acid-induced neurotoxicity.
10 Oct 2009
AIM: The present study has been designed to investigate the involvement of the nitric oxide mechanism in the protective effect of lycopene against 3-nitropropionic acid-induced Huntington's disease-like symptoms in rats. MAIN METHODS: The present ... Read more...
Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease.
21 Sep 2009
OBJECTIVE: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in the HD gene (HTT). We aimed to assess whether interaction between CAG repeat sizes in the mutant and normal allele could ... Read more...
14 Sep 2009
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, caused by a polyglutamine expansion in the huntingtin protein (htt). Increasing evidence suggests that transglutaminase (TGase) plays a critical role in the ... Read more...
Latest indexed articles for 'Huntington Disease'
These are the very latest articles for this heading:
- Neural grafts in Huntington's disease: viability after 10 years.
30 Oct 2009 - Lycopene modulates nitric oxide pathways against 3-nitropropionic acid-induced neurotoxicity.
10 Oct 2009 - Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease.
21 Sep 2009 - Huntington disease: a tale of two genes.
21 Sep 2009 - Striatal expression of a calmodulin fragment improved motor function, weight loss, and neuropathology in the R6/2 mouse model of Huntington's disease.
14 Sep 2009 - No evidence for disease-like processes in fetal transplants.
31 Aug 2009 - Interaction of postsynaptic density protein-95 with NMDA receptors influences excitotoxicity in the yeast artificial chromosome mouse model of Huntington's disease.
31 Aug 2009 - Allele-selective inhibition of mutant huntingtin by peptide nucleic acid-peptide conjugates, locked nucleic acid, and small interfering RNA.
30 Aug 2009 - Abstracts for the 2009 World Congress on Huntington's Disease, 12-15 September 2009, Vancouver, BC, Canada.
30 Aug 2009 - BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution.
25 Aug 2009 - Alterations in cortical excitation and inhibition in genetic mouse models of Huntington's disease.
17 Aug 2009 - Weight loss in Huntington disease increases with higher CAG repeat number.
16 Aug 2009 - Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons.
15 Aug 2009 - Cannabinoid CB2 receptor agonists protect the striatum against malonate toxicity: relevance for Huntington's disease.
13 Aug 2009 - Kaempferol protects against rat striatal degeneration induced by 3-nitropropionic acid.
11 Aug 2009 - A peek inside the Huntington's brain: will functional imaging take us one step closer in solving the puzzle?
8 Aug 2009 - Distinct neuroinflammatory profile in post-mortem human Huntington's disease.
3 Aug 2009 - Dietary intake in adults at risk for Huntington disease: analysis of PHAROS research participants.
2 Aug 2009 - [Differential diagnosis of chorea]
30 Jul 2009 - Huntington disease and other choreas.
30 Jul 2009
See a longer list of these articles.
Technical information about 'Huntington Disease'
Definition: A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
Descriptor UI: D006816
Alternative terms: Huntington Disease; Huntington Chorea; Chorea, Huntington; Huntington's Disease; Chronic Progressive Hereditary Chorea (Huntington); Huntington Chronic Progressive Hereditary Chorea; Progressive Chorea, Chronic Hereditary (Huntington); Progressive Chorea, Hereditary, Chronic (Huntington); Huntington's Chorea; Chorea, Huntington's; Chorea, Chronic Progressive Hereditary (Huntington); Huntington Disease, Late Onset; Late-Onset Huntington Disease; Huntington Disease, Late-Onset; Late Onset Huntington Disease; Juvenile Huntington Disease; Juvenile-Onset Huntington Disease; Juvenile Onset Huntington Disease; Huntington Disease, Juvenile-Onset; Huntington Disease, Juvenile Onset; Huntington Disease, Juvenile; Akinetic-Rigid Variant of Huntington Disease; Akinetic Rigid Variant of Huntington Disease; Huntington Disease, Akinetic-Rigid Variant; Huntington Disease, Akinetic Rigid Variant;
Allowable Qualifiers: blood; cerebrospinal fluid; chemically induced; classification; complications; diagnosis; diet therapy; drug therapy; economics; embryology; enzymology; ethnology; etiology; genetics; history; immunology; metabolism; microbiology; mortality; nursing; epidemiology; parasitology; pathology; physiopathology; prevention & control; psychology; radiography; radionuclide imaging; radiotherapy; rehabilitation; surgery; therapy; urine; veterinary; ultrasonography; virology;
Tree Number: C10.228.140.079.545; C10.228.140.380.278; C10.228.662.262.249.750; C10.574.500.497; C16.320.400.430; F03.087.250.400; F03.087.400.390;
History Note: 2000(1963)