RGD Reference Report - Transgenic rat model of Huntington's disease: a histopathological study and correlations with neurodegenerative process in the brain of HD patients. - Rat Genome Database

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Transgenic rat model of Huntington's disease: a histopathological study and correlations with neurodegenerative process in the brain of HD patients.

Authors: Mazurová, Yvona  Anderova, Miroslava  Nemecková, Ivana  Bezrouk, Aleš 
Citation: Mazurová Y, etal., Biomed Res Int. 2014;2014:291531. doi: 10.1155/2014/291531. Epub 2014 Aug 3.
RGD ID: 13452380
Pubmed: PMID:25162006   (View Abstract at PubMed)
PMCID: PMC4137602   (View Article at PubMed Central)
DOI: DOI:10.1155/2014/291531   (Journal Full-text)

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.

Phenotype Annotations    Click to see Annotation Detail View

Mammalian Phenotype

TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
neurodegeneration  IMP 13452380; 13452380 RGD 
Objects Annotated

Genes (Rattus norvegicus)
Htt  (huntingtin)

Strains
SD-Tg(Htt*)  (NA)


Additional Information