Huntington Disease

"Huntington Disease" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.

MeSH information

Definition | Details | More General Concepts | Related Concepts | More Specific Concepts

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 ID	D006816
MeSH Number(s)	C10.228.140.079.545 C10.228.140.380.278 C10.228.662.262.249.750 C10.574.500.497 C16.320.400.430 F03.615.250.400 F03.615.400.390
Concept/Terms	Huntington Disease 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 Huntington Disease, Late Onset Late-Onset Huntington Disease Huntington Disease, Late-Onset Late Onset Huntington Disease Juvenile 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 Akinetic Rigid Variant of Huntington Disease Huntington Disease, Akinetic-Rigid Variant Huntington Disease, Akinetic Rigid Variant

Below are MeSH descriptors whose meaning is more general than "Huntington Disease".

Diseases [C]
Nervous System Diseases [C10]
Central Nervous System Diseases [C10.228]
Brain Diseases [C10.228.140]
Basal Ganglia Diseases [C10.228.140.079]
Huntington Disease [C10.228.140.079.545]
Dementia [C10.228.140.380]
Huntington Disease [C10.228.140.380.278]
Movement Disorders [C10.228.662]
Dyskinesias [C10.228.662.262]
Chorea [C10.228.662.262.249]
Huntington Disease [C10.228.662.262.249.750]
Neurodegenerative Diseases [C10.574]
Heredodegenerative Disorders, Nervous System [C10.574.500]
Huntington Disease [C10.574.500.497]
Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
Genetic Diseases, Inborn [C16.320]
Heredodegenerative Disorders, Nervous System [C16.320.400]
Huntington Disease [C16.320.400.430]
Psychiatry and Psychology [F]
Mental Disorders [F03]
Neurocognitive Disorders [F03.615]
Cognition Disorders [F03.615.250]
Huntington Disease [F03.615.250.400]
Dementia [F03.615.400]
Huntington Disease [F03.615.400.390]

Below are MeSH descriptors whose meaning is related to "Huntington Disease".

Below are MeSH descriptors whose meaning is more specific than "Huntington Disease".

publications

Timeline | Most Recent

This graph shows the total number of publications written about "Huntington Disease" by people in this website by year, and whether "Huntington Disease" was a major or minor topic of these publications.

Bar chart showing 16 publications over 11 distinct years, with a maximum of 3 publications in 2007

To see the data from this visualization as text, click here.

Year	Major Topic	Minor Topic	Total
2001	2	0	2
2002	2	0	2
2003	0	1	1
2004	0	1	1
2005	2	0	2
2007	1	2	3
2008	1	0	1
2011	1	0	1
2014	1	0	1
2015	1	0	1
2021	0	1	1

Below are the most recent publications written about "Huntington Disease" by people in Profiles.

Pluciennik A, Liu Y, Molotsky E, Marsh GB, Ranxhi B, Arnold FJ, St-Cyr S, Davidson B, Pourshafie N, Lieberman AP, Gu W, Todi SV, Merry DE. Deubiquitinase USP7 contributes to the pathogenicity of spinal and bulbar muscular atrophy. J Clin Invest. 2021 01 04; 131(1).

View in: PubMed
Morozova OA, Gupta S, Colby DW. Prefibrillar huntingtin oligomers isolated from HD brain potently seed amyloid formation. FEBS Lett. 2015 Jul 08; 589(15):1897-903.

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Barbaro BA, Lukacsovich T, Agrawal N, Burke J, Bornemann DJ, Purcell JM, Worthge SA, Caricasole A, Weiss A, Song W, Morozova OA, Colby DW, Marsh JL. Comparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington's disease. Hum Mol Genet. 2015 Feb 15; 24(4):913-25.

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Gupta S, Jie S, Colby DW. Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay. J Biol Chem. 2012 Mar 23; 287(13):9982-9989.

View in: PubMed
Dragatsis I, Goldowitz D, Del Mar N, Deng YP, Meade CA, Liu L, Sun Z, Dietrich P, Yue J, Reiner A. CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse. Neurobiol Dis. 2009 Mar; 33(3):315-30.

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Reiner A, Del Mar N, Deng YP, Meade CA, Sun Z, Goldowitz D. R6/2 neurons with intranuclear inclusions survive for prolonged periods in the brains of chimeric mice. J Comp Neurol. 2007 Dec 20; 505(6):603-29.

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Gibb SL, Boston-Howes W, Lavina ZS, Gustincich S, Brown RH, Pasinelli P, Trotti D. A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis. J Biol Chem. 2007 Nov 02; 282(44):32480-90.

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Bogush A, Pedrini S, Pelta-Heller J, Chan T, Yang Q, Mao Z, Sluzas E, Gieringer T, Ehrlich ME. AKT and CDK5/p35 mediate brain-derived neurotrophic factor induction of DARPP-32 in medium size spiny neurons in vitro. J Biol Chem. 2007 Mar 09; 282(10):7352-9.

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Armen RS, Bernard BM, Day R, Alonso DO, Daggett V. Characterization of a possible amyloidogenic precursor in glutamine-repeat neurodegenerative diseases. Proc Natl Acad Sci U S A. 2005 Sep 20; 102(38):13433-8.

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Sun Z, Wang HB, Deng YP, Lei WL, Xie JP, Meade CA, Del Mar N, Goldowitz D, Reiner A. Increased calbindin-D28k immunoreactivity in striatal projection neurons of R6/2 Huntington's disease transgenic mice. Neurobiol Dis. 2005 Dec; 20(3):907-17.

View in: PubMed

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