发明名称 |
TRANSGENIC RAT AS ANIMAL MODEL FOR HUMAN HUNTINGDON'S DISEASE |
摘要 |
Huntingdon's Disease (HD) is an autosomal-dominant inherited progredient neurodegenerative disease from the group of CAG repeat/polyglutamine diseases and is characterized by a triad of psychiatric modifications, dementia and motory function disorders. On a sub-cellular level, a mutation with extended CAG trinucleotide repeats has been identified as the cause of Huntingdon's Disease. The therapeutic effects of certain substances can be tested on the neurochemically indicated transgenic animal models with expanded CAG repeats. In the present invention, transgenic rats were generated and characterized for human HD. Said rat model for human HD and other diseases of the CNS includes 51 CAG repeats under the control of a rat promoter and has a slowly progredient neurological phenotype closely reflecting human HD syndrome. The comparability of the rat model in relation to human HD is characterized by neuropathological, neuroradiological and neurochemical modifications accompanied by typical behavioural abnormalities. |
申请公布号 |
WO03095640(A3) |
申请公布日期 |
2004.01.22 |
申请号 |
WO2003DE01546 |
申请日期 |
2003.05.14 |
申请人 |
MEDIZINISCHE HOCHSCHULE HANNOVER;RIESS, OLAF;VON HOERSTEN, STEPHAN |
发明人 |
RIESS, OLAF;VON HOERSTEN, STEPHAN |
分类号 |
A01K67/027;C07K14/47;C12N15/85 |
主分类号 |
A01K67/027 |
代理机构 |
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代理人 |
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主权项 |
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地址 |
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