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IV: Models and Applications

IV: Models and Applications 70 AAMBR 2010 abstracts PLENARY LECTURES The Drosophila Homologue of Ataxin-2 Binding Protein: Toward a Fruit Fly Model of Spinocerebellar Ataxia Type 2? 1 1,2 N. Usha and L.S. Shashidhara* E-mail: ls.shashidhara@iiserpune.ac.in Centre for Cellular and Molecular Biology (CSIR), Hyderabad 500007, Andhra Pradesh, India Indian Institute of Science Education and Research, Pune 411008, Maharastra, India Expansion of polyglutamine repeats in Ataxin-2 causes spinocerebellar ataxia type 2 (SCA2), a neurodegenerative disease. Ataxin-2 activity is modulated by the Ataxin-2 Binding Protein 1 (A2BP1). We have observed that the Drosophila homologue of A2BP1 (dA2BP1) functions as a co-factor of Cubitus interruptus (Ci; its human homologue is Gli, the sole transducer of Sonic hedgehog activity). Specifically, Ci-mediated trans-activation of knot/collier (kn) is dependent on dA2BP1 function. Protein interaction studies and chromatin-immunoprecipitation experi- ments suggest that Ci helps dA2BP1 to bind to the kn promoter, which in turn may help Ci to activate kn expression. Interestingly, early B-cell factor 2 (EBF2), a target of Gli, is mam- malian homologue of Drosophila Knot/Collier. Ebf2 is required for the establishment of a proper cerebellar cortical map. Ebf2-null mice display ataxia phenotypes and motor deficits associated with abnormalities of cerebellar development. In this context, our results on http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Alternatives to Laboratory Animals SAGE

IV: Models and Applications

Alternatives to Laboratory Animals , Volume 39 (1): 3 – Mar 1, 2011
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Publisher
SAGE
Copyright
© 2011 Fund for the Replacement of Animals in Medical Experiments
ISSN
0261-1929
eISSN
2632-3559
DOI
10.1177/026119291103900106
Publisher site
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Abstract

70 AAMBR 2010 abstracts PLENARY LECTURES The Drosophila Homologue of Ataxin-2 Binding Protein: Toward a Fruit Fly Model of Spinocerebellar Ataxia Type 2? 1 1,2 N. Usha and L.S. Shashidhara* E-mail: ls.shashidhara@iiserpune.ac.in Centre for Cellular and Molecular Biology (CSIR), Hyderabad 500007, Andhra Pradesh, India Indian Institute of Science Education and Research, Pune 411008, Maharastra, India Expansion of polyglutamine repeats in Ataxin-2 causes spinocerebellar ataxia type 2 (SCA2), a neurodegenerative disease. Ataxin-2 activity is modulated by the Ataxin-2 Binding Protein 1 (A2BP1). We have observed that the Drosophila homologue of A2BP1 (dA2BP1) functions as a co-factor of Cubitus interruptus (Ci; its human homologue is Gli, the sole transducer of Sonic hedgehog activity). Specifically, Ci-mediated trans-activation of knot/collier (kn) is dependent on dA2BP1 function. Protein interaction studies and chromatin-immunoprecipitation experi- ments suggest that Ci helps dA2BP1 to bind to the kn promoter, which in turn may help Ci to activate kn expression. Interestingly, early B-cell factor 2 (EBF2), a target of Gli, is mam- malian homologue of Drosophila Knot/Collier. Ebf2 is required for the establishment of a proper cerebellar cortical map. Ebf2-null mice display ataxia phenotypes and motor deficits associated with abnormalities of cerebellar development. In this context, our results on

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Alternatives to Laboratory Animals – SAGE

Published: Mar 1, 2011

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