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Article
Open AccessThe length of uninterrupted CAG repeats in stem regions of repeat disease associated hairpins determines the amount of short CAG oligonucleotides that are toxic to cells through RNA interference
Extended CAG trinucleotide repeats (TNR) in the genes huntingtin (HTT) and androgen receptor (AR) are the cause of two progressive neurodegenerative disorders: Huntington’s disease (HD) and Spinal and Bulbar Musc...
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Article
Open AccessCharacterization of microtubule-associated protein tau isoforms and Alzheimer’s disease-like pathology in normal sheep (Ovis aries): relevance to their potential as a model of Alzheimer’s disease
Alzheimer’s disease is a chronic neurodegenerative disease that accounts for up to 80% of all dementias. Characterised by deteriorations of memory and cognitive function, the key neuropathological features are...
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Article
Open AccessWake-Promoting and EEG Spectral Effects of Modafinil After Acute or Chronic Administration in the R6/2 Mouse Model of Huntington’s Disease
Huntington’s disease (HD) is characterised by progressive symptoms including cognitive deficits and sleep/wake disturbances reflected in an abnormal electroencephalography (EEG). Modafinil, a wake-promoting an...
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Article
Open AccessIndices of comparative cognition: assessing animal models of human brain function
Understanding the cognitive capacities of animals is important, because (a) several animal models of human neurodegenerative disease are considered poor representatives of the human equivalent and (b) cognitiv...
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Article
Erratum to: Chronic Paroxetine Treatment Prevents the Emergence of Abnormal Electroencephalogram Oscillations in Huntington’s Disease Mice
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Open AccessChronic Paroxetine Treatment Prevents the Emergence of Abnormal Electroencephalogram Oscillations in Huntington’s Disease Mice
Disturbance of rapid eye movement (REM) sleep appears early in both patients with Huntington’s disease (HD) and mouse models of HD. Selective serotonin reuptake inhibitors are widely prescribed for patients wi...
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Article
Open AccessA stop-signal task for sheep: introduction and validation of a direct measure for the stop-signal reaction time
Huntington’s disease (HD) patients show reduced flexibility in inhibiting an already-started response. This can be quantified by the stop-signal task. The aim of this study was to develop and validate a sheep...
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Article
Open AccessMetabolic profiling of presymptomatic Huntington’s disease sheep reveals novel biomarkers
The pronounced cachexia (unexplained wasting) seen in Huntington’s disease (HD) patients suggests that metabolic dysregulation plays a role in HD pathogenesis, although evidence of metabolic abnormalities in H...
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Article
Open AccessPotential molecular consequences of transgene integration: The R6/2 mouse example
Integration of exogenous DNA into a host genome represents an important route to generate animal and cellular models for exploration into human disease and therapeutic development. In most models, little is kn...
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Article
Open AccessImpaired Nitric Oxide Mediated Vasodilation In The Peripheral Circulation In The R6/2 Mouse Model Of Huntington’s Disease
Recent evidence shows that the Huntington’s disease (HD) extends beyond the nervous system to other sites, including the cardiovascular system. Further, the cardiovascular pathology pre-dates neurological decl...
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Article
Choosing an animal model for the study of Huntington's disease
Animal models of Huntington's disease (HD), which have been established in species that range from worms, fruitflies, mice and rats to pigs, sheep and monkeys,...
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Article
Determining association networks in social animals: choosing spatial–temporal criteria and sampling rates
Social Network Analysis has become an important methodological tool for advancing our understanding of human and animal group behaviour. However, researchers tend to rely on arbitrary distance and time measure...
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Article
Asymptomatic Sleep Abnormalities Are a Common Early Feature in Patients with Huntington’s Disease
Huntington’s disease (HD) is a fatal neurodegenerative disease characterized by motor, cognitive, and psychiatric disturbance. In this article, we used polysomnography, actigraphy and a variety of validated qu...
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Synaptic Dysfunction in Huntington’s Disease
Huntington’s disease (HD) is a progressive, inherited, neurodegenerative disorder characterised by movement abnormalities, cognitive impairments and emotional disturbance (Bates et al. 2002). The genetic mutation...
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Article
Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington’s disease
The histological hallmark feature of Huntington’s disease (HD) and other polyglutamine repeat diseases is the presence of intracellular inclusions. Much work has been devoted to trying to determine the relatio...
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Article
Measuring cognitive deficits in disabled mice using an automated interactive touchscreen system
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Article
Atypical diabetes associated with inclusion formation in the R6/2 mouse model of Huntington’s disease is not improved by treatment with hypoglycaemic agents
The R6/2 transgenic mouse model of Huntington’s disease (HD) develops a progressive neurological phenotype that involves severe motor and cognitive dysfunctions. Although not a cardinal sign, diabetes has been...
