I. INTRODUCTION 1 1.1 Cell death in SOD1G93A-linked ALS 1 1.2 Cell death in TDP43Q331K-linked ALS 1 1.3 Cell death in C9Orf72PR50-linked ALS 2 1.4 Current treatment for ALS 3 II. MATERIALS AND METHODS 6 2.1 Construction of expression vectors 6 2.2 In ovo electroporation 6 2.3 Tissue preparation for immunostaining 6 2.4 RNA In situ Hybridization 7 2.5 Immunohistochemistry and confocal Imaging 8 2.6 Image and Statistical analysis 8 III. RESULTS 10 3.1 Embryonic chicken as ALS model and mRNA expression in spinal cord neuron 10 3.2 mRNA expression in Tfam electroporated spinal cord 11 3.3 mRNA expression in Gpld1 electroporated spinal cord 12 3.4 Caspase-3 positive cells in different ALS mutants 15 3.5 Protective effects of Tfam in ALS mutants 15 3.6 Protective effects of Gpld1 in ALS mutants 18 IV. DISCUSSION 19 V. CONCLUSION 21 VI. REFERENCES 22 요약문 27
