Decrease of Parkin and CD36 expression and body weight correlate with an increase of non-esterified fatty acids in a mouse model for TDP-43 pathology

TDP-43-coding mutations are found among ALS patients. In order to express human mutant TDP-43 protein in the mouse under the control of the endogenous Tardp promoter, we have generated a mouse line with 300% elevated TDP-43 level. Total RNA obtained from 4-6 male mutant mice was compared to 4-6 wild type controls.TAR DNA结合蛋白43 (TDP-43) 编码突变可在肌萎缩侧索硬化 (ALS) 患者中检出。为在内源Tardp启动子的调控下于小鼠体内表达人源突变型TDP-43蛋白，我们构建了TDP-43表达水平升高300%的小鼠品系。我们提取了4~6只雄性突变小鼠的总RNA，并与4~6只野生型对照小鼠的总RNA进行比对分析。