title: Early onset deficits on the delayed alternation task in the Hdh(Q92) knock-in mouse model of Huntington's disease.
abstract: A number of genetic mouse models of Huntington's disease have been created, in order to examine the pathogenesis of Huntington's disease and to test potential therapeutics. In the present study we demonstrate that the full-length knock-in homozygote Hdh(Q92) mice exhibit impairments at 5 months of age on the delayed alternation task, conducted in 9-hole operant chambers. This test is sensitive to cortico-striatal dysfunction and demonstrates again that although Hdh(Q92) mice do not display an overt motor phenotype, they do exhibit clear impairments that can be related to deficits seen in HD patients. This indicates that if appropriately sensitive tasks are used, the more subtle and specific Hdh(Q92) knock-in model could be of use for the examination of pathogenic mechanisms in Huntington's disease and to test potential therapeutics.
authors: Brain Repair Group, School of Biosciences, Cardiff University, Wales, UK. TruemanRC@cf.ac.uk
authors: R C
authors: RC
authors: Trueman
authors: L
authors: L
authors: Jones
authors: S B
authors: SB
authors: Dunnett
authors: S P
authors: SP
authors: Brooks
mesh_terms: D017668:Age of Onset; D000818:Animals; D003342:Corpus Striatum / Q000503:physiopathology; D004195:Disease Models, Animal; D005260:Female; D055879:Gene Knock-In Techniques; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology*; D007859:Learning Disabilities / Q000209:etiology / Q000235:genetics; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009043:Motor Activity / Q000502:physiology; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism*; D011930:Reaction Time / Q000235:genetics; D045442:Rotarod Performance Test
chemical_list: C506812:Htt protein, mouse; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins