title: Transgenic rat model of Huntington's disease: a histopathological study and correlations with neurodegenerative process in the brain of HD patients.
abstract: Rats transgenic for Huntington's disease (tgHD51 CAG rats), surviving up to two years, represent an animal model of HD similar to the late-onset form of human disease. This enables us to follow histopathological changes in course of neurodegenerative process (NDP) within the striatum and compare them with postmortem samples of human HD brains. A basic difference between HD pathology in human and tgHD51 rats is in the rate of NDP progression that originates primarily from slow neuronal degeneration consequently resulting in lesser extent of concomitant reactive gliosis in the brain of tgHD51 rats. Although larger amount of striatal neurons displays only gradual decrease in their size, their number is significantly reduced in the oldest tgHD51 rats. Our quantitative analysis proved that the end of the first year represents the turn in the development of morphological changes related to the progression of NDP in tgHD51 rats. Our data also support the view that all types of CNS glial cells play an important, irreplaceable role in NDP. To the best of our knowledge, our findings are the first to document that tgHD51 CAG rats can be used as a valid animal model for detailed histopathological studies related to HD in human.
authors.affiliation: Department of Histology and Embryology, Faculty of Medicine in Hradec Králové, Charles University in Prague, Šimkova 870, P.O. Box 38, 500 38 Hradec Králové, Czech Republic.
authors.forename: Yvona
authors.initials: Y
authors.lastname: Mazurová
authors.affiliation: Department of Cellular Neurophysiology, Institute of Experimental Medicine, The Academy of Sciences, Vídeňská 1083, 142 20 Prague, Czech Republic.
authors.forename: Miroslava
authors.identifier: 0000-0002-9030-4571
authors.initials: M
authors.lastname: Anderova
authors.affiliation: Department of Biological and Medical Sciences, Faculty of Pharmacy in Hradec Králové, Charles University in Prague, Heyrovského 1203, 500 05 Hradec Králové, Czech Republic.
authors.forename: Ivana
authors.identifier: 0000-0002-9795-8471
authors.initials: I
authors.lastname: Němečková
authors.affiliation: Department of Medical Biophysics, Faculty of Medicine in Hradec Králové, Charles University in Prague, Šimkova 870, P.O. Box 38, 500 38 Hradec Králové, Czech Republic.
authors.forename: Aleš
authors.identifier: 0000-0002-2397-3847
authors.initials: A
authors.lastname: Bezrouk
mesh_terms: D000818:Animals; D003342:Corpus Striatum / Q000473:pathology*; D004195:Disease Models, Animal; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000473:pathology*; D009410:Nerve Degeneration / Q000235:genetics / Q000473:pathology*; D009474:Neurons / Q000473:pathology; D051381:Rats; D055647:Rats, Transgenic; D019680:Trinucleotide Repeat Expansion / Q000235:genetics*