Experimental models of Huntington's disease

Introduction. Huntington's disease (HD) is an autosomal dominant hereditary disease caused by triplet repetition in exon I of the huntingtin protein located in chromosome 4. Medium spiny neurons in the striatum are selectively affected. Clinical manifestations include progressive behavioural, motor and cognitive disorders. There is no treatment available today capable of modifying the natural course of the disease. A great amount of research work is being carried out, much of which involves animal models of the disease. Development. We reviewed the articles published in PubMed on basic research into HD and analysed the most frequently used models. Transgenic mouse models, excitotoxic models, transgenic fly models and cell cultures are all used in studies into HD. The advantages and disadvantages of each of them are highlighted. Conclusion. The contribution made by each model of HD must be known in order to draw up a correct design in experimental studies of the disease. [REV NEUROL 2007; 45: 437-41]