Methods and compositions for the identification and...

A - Human Necessities – 61 – K

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A61K 48/00 (2006.01) A01K 67/033 (2006.01) A61K 31/7088 (2006.01) A61K 38/00 (2006.01) A61K 39/12 (2006.01) A61K 39/395 (2006.01) A61P 25/28 (2006.01) A61P 35/00 (2006.01) C07K 14/47 (2006.01) C12Q 1/00 (2006.01) C12Q 1/68 (2006.01)

Patent

CA 2427061

The present invention relates to Drosophila models of the neurodegenerative disorder spinocerebellar ataxia 1 (SCA-1). In particular, the invention relates to transgenic Drosophila which express normal human ataxin-1 or mutant human ataxin-1 with expanded polyglutamine repeats for SCA-1 therapeutics. The invention further relates to the diagnosis of predispositions to developing SCA-1. The invention further relates to methods of using the transgenic Drosophila to screen for therapeutics of SCA-1 and other neurodegenerative disorders. The invention further relates to the identification of modifier genes of the SCA-1 phenotypes produced by overexpression of ataxin-1, for therapeutic and diagnostic uses and for screening for therapeutics of SCA-1 and other neurodegenerative disorders. The invention further relates to the diagnosis of a predisposition to SCA-1 comprising detecting the overexpression of normal ataxin-1.

La présente invention concerne des modèles de mouche drosophile représentant l'ataxie spino-cérebelleuse 1 (SCA-1), un trouble neurodégénératif (SCA-1). Notamment, cette invention a trait à une mouche drosophile transgénique exprimant la protéine ataxine-1 ou la protéine ataxine-1 humaine mutante avec des séquences nucléotidiques répétées de polyglutamine dilatée utilisée dans les thérapies pour SCA-1. Cette invention a aussi trait au diagnostic de prédispositions au développement de SCA-1, ainsi que des méthodes d'utilisation de la mouche drosophile transgénique de manière à rechercher les thérapies pour SCA-1 et autres troubles neurodégénératifs. Cette invention concerne aussi l'identification de gènes modificateurs des phénotypes SCA-1 produits par surexpression de la protéine d'ataxine-1, dans le cadre d'utilisations thérapeutiques et diagnostiques et dans la recherche de thérapies pour SCA-1 et autres troubles neurodégénératifs. Ladite invention a aussi trait au diagnostic d'un prédisposition à SCA-1 consistant à déceler la surexpression de l'ataxine-1 normale.

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