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Huntington disease


'Huntington disease' can also refer to...

The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin–proteasome system

Aberrant cortical synaptic plasticity and dopaminergic dysfunction in a mouse model of huntington's disease

Aberrant Interactions of Transcriptional Repressor Proteins with the Huntington's Disease Gene Product, Huntingtin

Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage

Abnormalities in the functioning of adipocytes from R6/2 mice that are transgenic for the Huntington’s disease mutation

Absence of Disease Phenotype and Intergenerational Stability of the Cag Repeat in Transgenic Mice Expressing the Human Huntington Disease Transcript

Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease

Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models

AKT-sensitive or insensitive pathways of toxicity in glial cells and neurons in Drosophila models of Huntington's disease

Altered Manganese Homeostasis and Manganese Toxicity in a Huntington's Disease Striatal Cell Model Are Not Explained by Defects in the Iron Transport System

Altered serotonin receptor expression is associated with depression-related behavior in the R6/1 transgenic mouse model of Huntington's disease

Analysis of Germline Mutation Spectra at the Huntington's Disease Locus Supports a Mitotic Mutation Mechanism

Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington's disease

Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease

Blocking acid-sensing ion channel 1 alleviates Huntington's disease pathology via an ubiquitin-proteasome system-dependent mechanism

CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease

Calpain and STriatal-Enriched protein tyrosine Phosphatase (STEP) activation contribute to extrasynaptic NMDA receptor localization in a Huntington's disease mouse model

Cell loss in the motor and cingulate cortex correlates with symptomatology in Huntington’s disease

A Cellular Model That Recapitulates Major Pathogenic Steps of Huntington's Disease

Central motor processing in Huntington's disease. A PET study.

Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington’s disease (HD) transgenic mice and HD patients

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity

Cholinergic neuronal defect without cell loss in Huntington's disease

Chromosomal profiles of gene expression in Huntington's disease

Clinical features of early and juvenile onset in polyglutamine disorders other than Huntington’s disease: autosomal dominant cerebellar ataxias and dentatorubral pallidoluysian atrophy

The clinical phenotype of juvenile Huntington’s disease

Cognitive deficits in Huntington's disease are predicted by dopaminergic PET markers and brain volumes.

Compensatory changes in the ubiquitin–proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice partially model Huntington's disease patients

Contribution of DNA Sequence and CAG Size to Mutation Frequencies of Intermediate Alleles for Huntington Disease: Evidence from Single Sperm Analyses

Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease

 

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Quick Reference

(Huntington’s chorea) a hereditary disease caused by a defect in a single gene that is inherited as an autosomal dominant characteristic, therefore tending to appear in half of the children of the parents with this condition. Symptoms, which begin to appear in early middle age, include unsteady gait and jerky involuntary movements (see chorea), accompanied later by behavioural changes, and progressive dementia. There is a defect in the Huntington gene on chromosome 4; genetic screening is possible for those at risk. G. Huntington (1850–1916), US physician

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