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TRANSGENIC RAT AND THE USE THEREOF IN THE ANIMAL MODEL FOR HUMAN HUNTINGDON'S DISEASE AND NUCLEAR CONSTRUCTS, VECTORS AND CELLS FOR THE PRODUCTION THEREOF
TRANSGENIC RAT AND THE USE THEREOF IN THE ANIMAL MODEL FOR HUMAN HUNTINGDON'S DISEASE AND NUCLEAR CONSTRUCTS, VECTORS AND CELLS FOR THE PRODUCTION THEREOF
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机译:转基因大鼠及其在动物模型中的应用(人类亨廷顿氏病和核构造,矢量和细胞)
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摘要
Huntingdon's Disease (HD) is an autosomal-dominant inherited progredientneurodegenerative disease from the group of CAG repeat/polyglutamine diseasesand is characterized by a triad of psychiatric modifications, dementia andmotory function disorders. On a sub-cellular level, a mutation with extendedCAG trinucleotide repeats has been identified as the cause of Huntingdon'sDisease. The therapeutic effects of certain substances can be tested on theneurochemically indicated transgenic animal models with expanded CAG repeats.In the present invention, transgenic rats were generated and characterized forhuman HD. Said rat model for human HD and other diseases of the CNS includes51 CAG repeats under the control of a rat promoter and has a slowlyprogredient neurological phenotype closely reflecting human HD syndrome. Thecomparability of the rat model in relation to human HD is characterized byneuropathological, neuroradiological and neurochemical modificationsaccompanied by typical behavioural abnormalities.
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