Ataxin-2 and FMRP have been known the most important factor in neurodegenerative, neurodevelopmental disease. However, it is very difficult to specify the function of Ataxin-2 and FMRP because of the various disease types. Although recent study reported that a direct interaction between Ataxin-2 and FMRP, the specific function and regulatory pathway are elusive. Interestingly, we found the increased amount of Ataxin-2 causes dysregulation of FMRP and defects of dendrite morphology in highly complicated neuron. Furthermore, we observed these phenomena can be occurred depending on PABP or motor protein, which is most popular binding partner of Ataxin-2 and FMRP. These results suggest that a specific regulatory mechanism of FMRP with Ataxin-2-PABP complex, and we expected that it contributes to reveal the detailed interaction of Ataxin-2, PABP, and FMRP.
Table Of Contents
Abstract i List of Contents ii List of Figures iii List of Tables iv
I. INTRODUCTION 1 1.1 Representative function of Ataxin-2 and its relationship with neurodegenerative diseases 1 1.2 Representative function of FMRP and Fragile X associated disorder and diseases 2 1.3 Interaction of Ataxin-2 and FMRP 2 II. MATERIALS AND METHODS 5 III. RESULTS 8 3.1 Reducing tendency of endogenous or exogenous expressed dFMR1 is observed in dATX2 overexpression flies 8 3.2 Overexpression of dATX2 induces dysregulation of dFMR1 and severe defects of dendrite morphology in Drosophila class IV da neuron 15 3.3 Dysregulation of dFMR1 and dendrite defects can be associated with PABP and motor proteins 18 IV. DISCUSSION 25 V. REFERENCES 27 VI. SUMMARY (국문요약) 29