Machado-Joseph disease (MJD) is an inherited neurodegenerative disorder caused by the expansion of the polyglutamine stretch in the MJD gene-encoded protein, ataxin-3. Using a series of deletion constructs expressing ataxin-3 fragments with expanded polyglutamine stretches, we observed aggregate formation and cell death in cultured BHK-21 cells. The cytotoxic effect of N-terminal-truncated ataxin-3 with the expanded polyglutamine tract was enhanced under serum starvation culture, in which cells were arrested in the G(0)/G(1)phase. Coexpression of p21 (waf1/cip1/sdi1), a cyclin-Cdk inhibitor that induced cell cycle arrest in the G(1)phase, also increased the cell death susceptibility produced by the mutant ataxin-3 fragment in BHK-21 cells. The elevated susceptibility to cell death in the G(0)/G(1)phase was confirmed in nerve growth factor-treated, postmitotic neuronal PC12 cells compared with undifferentiated proliferating PC12 cells. These results strongly suggest that the cellular toxicity of truncated ataxin-3 with an expanded polyglutamine stretch is enhanced by cell cycle arrest in the G(0)/G(1)phase. Mutant ataxin-3 may confer a higher susceptibility to cell death on cells in the G(0)/G(1)phase.

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This document intends to describe a system overview of "Integrated Broadband Environment for Personalized TV Experience (IBEX)". The IBEX features three major goals: to release audience from time constraint scheduled by broadcasters, to provide seamless and adaptive access between TV and the Web, and finally, to securely personalize TV experience for individual audience. In order to achieve open framework, these viewpoints must be properly developed in conjunction with well-organized data models and system architecture based on de*facto standard technology. The IBEX will provide a prototype system model built on upcoming integrated broadband digital TV infrastructure. The IBEX also intends to provide a service model for the TV-Anytime framework.

includes from TV broadcasters to TV manufacturers. Core parts of the specifications, Metadata and Content Referencing, are ready This is a continuing work on the IBEX, that is Integrated Broadband Enviromnent for Personalized TV Experience. In this paper, we describe our implementation study of the IBEX as a TV-Anytime service platform. We also describe potential IBEX applications on the basis of TV-Anytime specifications. The Adaptive Content Guide (ACG) is expected to provide consumers with adaptive access to favorite contents and metadata customized not only for user preferences but also terminal capabilities. We also found current TV-Anytime specification lacks ability of describing terminal capabilities, which is required for universal for use and implementation studies are strongly desired in order to prove the feasibility of the standard.In this paper, we describe our implementation study of the "Integrated Broadband Environment for Personalized TV Experience (IBEX)"[6] as a TV-Anytime service platform, succeeding our previous service-oriented demonstration for TV-Anytime Forum at NAB2001. We also describe potential IBEX applications on the basis of TV-Anytime Specifications. SYSTEM MODELaccess to the ACG.

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