Correlations Between Mutant Huntingtin Aggregates and Behavioral Changes in R6/1 Mice.

Background: Huntington's disease (HD) is a neurodegenerative disorder caused by the expansion of the trinucleotide CAG in the HD gene. While the presence of nuclear aggregates of mutant huntingtin (mHtt) in neurons is a hallmark of HD, the reason behind its toxicity remains elusive.

Objective: The present study was conducted to assess a correlation between the number of mHtt aggregates and the severity of HD symptoms in R6/1 mice.

Neurophysiological and Behavioral Effects of Anti-Orexinergic Treatments in a Mouse Model of Huntington's Disease.

Huntington's disease (HD) is associated with sleep and circadian disturbances in addition to hallmark motor and cognitive impairments. Electrophysiological studies on HD mouse models have revealed an aberrant oscillatory activity at the beta frequency, during sleep, that is associated with HD pathology. Moreover, HD animal models display an abnormal sleep-wake cycle and sleep fragmentation.

3-D motion capture for long-term tracking of spontaneous locomotor behaviors and circadian sleep/wake rhythms in mouse.

Background: Locomotor activity provides an index of an animal's behavioral state. Here, we report a reliable and cost-effective method that allows long-term (days to months) simultaneous tracking of locomotion in mouse cohorts (here consisting of 24 animals).

New Method: The technique is based on a motion capture system used mainly for human movement study.

Changes in striatal activity and functional connectivity in a mouse model of Huntington's disease.

Hereditary Huntington's disease (HD) is associated with progressive motor, cognitive and psychiatric symptoms. A primary consequence of the HD mutation is the preferential loss of medium spiny projection cells with relative sparing of local interneurons in the striatum. In addition, among GABAergic striatal projection cells, indirect pathway cells expressing D2 dopamine receptors are lost earlier than direct pathway cells expressing D1 receptors.

Sustained Gq-Protein Signaling Disrupts Striatal Circuits via JNK.

Unlabelled: The dorsal striatum is a major input structure of the basal ganglia and plays a key role in the control of vital processes such as motor behavior, cognition, and motivation. The functionality of striatal neurons is tightly controlled by various metabotropic receptors. Whereas the G/G-protein-dependent tuning of striatal neurons is fairly well known, the precise impact and underlying mechanism of G-protein-dependent signals remain poorly understood.

Chronic cannabinoid receptor stimulation selectively prevents motor impairments in a mouse model of Huntington's disease.

Huntington's disease (HD) is a devastating neurodegenerative disease characterized by a progressive decline in motor abilities, as well as in cognitive and social behaviors. Most of these behavioral deficits are recapitulated in the R6/1 transgenic mouse, which can therefore be used as an experimental model to identify the neurobiological substrates of HD pathology and to design novel therapeutic approaches. The endocannabinoid system (ECS) is a relevant candidate to participate in the etiopathology of HD as it is a key modulator of brain function, especially in areas primarily affected by HD dysfunction such as the striatum.