Huntington Disease - Research News and Information

Saturday, January 10, 2009

Weight loss in Huntington disease increases with higher CAG repeat number.

2 Nov 2008 OBJECTIVE: Huntington disease (HD) is a hereditary neurodegenerative disorder caused by an expanded number of CAG repeats in the huntingtin gene. A hallmark of HD is unintended weight loss, the cause of which is unknown. In order to elucidate the ... Read more...

Linking SNPs to CAG repeat length in Huntington's disease patients.

17 Oct 2008 Allele-specific silencing using small interfering RNAs targeting heterozygous single-nucleotide polymorphisms (SNPs) is a promising therapy for human trinucleotide repeat diseases such as Huntington's disease. Linking SNP identities to the two HTT ... Read more...

Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner.

13 Oct 2008 Transcriptional dysregulation is a central pathogenic mechanism in Huntington's disease, a fatal neurodegenerative disorder associated with polyglutamine (polyQ) expansion in the huntingtin (Htt) protein. In this study, we show that mutant Htt ... Read more...

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Latest indexed articles for 'Huntington Disease'

These are the very latest articles for this heading:

• Weight loss in Huntington disease increases with higher CAG repeat number. 2 Nov 2008
• Linking SNPs to CAG repeat length in Huntington's disease patients. 17 Oct 2008
• Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner. 13 Oct 2008
• [Huntington's disease] 7 Oct 2008
• [Artistic creativeness and Huntington disease] 7 Oct 2008
• Abstracts of the EHDN (European Huntington Disease Network) Annual Meeting, September 4-7, 2008. Lisbon, Portugal. 29 Sep 2008
• Dopaminergic and glutamatergic signaling crosstalk in Huntington's disease neurodegeneration: the role of p25/cyclin-dependent kinase 5. 29 Sep 2008
• Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry. 22 Sep 2008
• Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease. 15 Sep 2008
• Predictive genetic testing of adolescents for Huntington disease: a question of autonomy and harm. 13 Sep 2008
• Altered information processing in the prefrontal cortex of Huntington's disease mouse models. Sep 2008
• Drug discovery and development for Huntington's disease - an orphan indication with high medical need. 30 Aug 2008
• Phonemic fluency deficits in asymptomatic gene carriers for Huntington's disease. 30 Aug 2008
• Aripiprazole effects on psychosis and chorea in a patient with Huntington's disease. 30 Aug 2008
• Role of copper in human neurological disorders. 30 Aug 2008
• Reduction in cerebral atrophy associated with ethyl-eicosapentaenoic acid treatment in patients with Huntington's disease. 30 Aug 2008
• Towards humane end points: behavioural changes precede clinical signs of disease in a Huntington's disease model. 20 Aug 2008
• Increased glucose metabolism and ATP level in brain tissue of Huntington's disease transgenic mice. 19 Aug 2008
• Protective role of Engrailed in a Drosophila model of Huntington's disease. 18 Aug 2008
• Fear of health insurance loss among individuals at risk for Huntington disease. 13 Aug 2008

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)