Beal, M.F., and Ferrante, R.J., 2004, Experimental therapeutics in transgenic mouse models of Huntington’s disease. Nat. Rev. Neurosci. 5: 373–384.Beal MF, Ferrante RJ (2004) Experimental therapeutics in tran
Neurodegenerative disease: Histone deacetylase 4 promotes cytoplasmic huntingtin aggregation in mouse models of Huntington diseaseIn the brains of mice with Huntington disease (HD), the transcriptional repressor histone deacetylase 4 (HDAC4) colocalizes with mutant huntingtin to form cytoplasmic inclusions,...
In this review, we provide an overview of the current status, uses and limitations of genetic mouse models for understanding major neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's disease and amyotrophic lateral sclerosis....
Huntington's Disease Mouse models Excitotoxicity Neurodegeneration Polyglutamine disease Protein aggregation Behavior 1. Introduction HDis an inheritedneurodegenerative diseasewith fatal prognosis characterized by progressive motor, cognitive and psychiatric impairments that develop near middle age, with most patients...
assessment of potential therapeutics should aid in the translation of new treatments. Here, we set out to determine how the concentrations of these three proteins change in plasma and CSF with disease progression in representative, well-established mouse models of Huntington’s disease. Plasma and ...
common feature in mouse models [15–16,19–21]. The cardiac-specific expression of polyQ repeats leads to dysfunction, suggesting that cardiovascular disease is a result of both local dys- function as well as improper input [22, 23]. Advances in these preclinical models are critical if we ar...
Huntington's disease (HD) is a monogenetic, neurodegenerative disease. It is fatal, and although treatments are available for minor symptomatic relief, it remains incurable. Careful study of models...doi:10.1007/978-1-61779-301-1_1Miriam A. Hickey...
intrabodies that had been shown, in cell cultures and fruit-fly models, to reduce the accumulation of toxic Htt protein. To see whether those effects would hold true in mammalian systems as well, the team tested the intrabodies in a series of five different mouse models of Huntington's. ...
Acta Neuropathologica Communications https://doi.org/10.1186/s40478-020-0878-0 (2020) 8:14 RESEARCH Open Access Thalamostriatal degeneration contributes to dystonia and cholinergic interneuron dysfunction in a mouse model of Huntington's disease Gabriel Crevier-Sorbo, Vladimir V. Rymar, Raphael ...
However, recent studies have stablished that environmental factors such as physical activity have a significant impact in the progression of the disease [10]. For example, in mouse models of HD, physical training seems to decrease protein aggregation, cell death and mitochondrial dysfunction. More...