【摘 要】
：
Mutations in the Fused in sarcoma/Translated in liposarcoma gene (FUS/TLS,FUS) have been identified among patients with amyotrophic lateral sclerosis (ALS).FUS
【机 构】
：
National Laboratory of Medical Molecular Biology, Institute of Basic Medical Sciences, Chinese Acade
论文部分内容阅读
Mutations in the Fused in sarcoma/Translated in liposarcoma gene (FUS/TLS,FUS) have been identified among patients with amyotrophic lateral sclerosis (ALS).FUS protein aggregation is a major pathological hallmark of FUS proteinopathy,a group of neurodegenerative diseases characterized by FUS-immunoreactive inclusion bodies.We prepared transgenic Drosophila expressing either the wild type (Wt) or ALS-mutant human FUS protein (hFUS) using the UAS-Gal4 system.When expressing Wt,R524S or P525L mutant FUS in photoreceptors,mushroom bodies (MBs) or motor neurons (MNs),transgenic flies show age-dependent progressive neural damages,including axonal loss in MB neurons,morphological changes and functional impairment in MNs.The transgenic flies expressing the hFUS gene recapitulate key features of FUS proteinopathy,representing the first stable animal model for this group of devastating diseases.
其他文献
大豆新品种广岛火红１号大豆新品种广岛火红１号是１９８８年８月由中科院的科技人员和湖北农科院的科技人员访日时，由广岛市火红农科所赠送给我国的优秀大豆品种。该品种产量高，早熟，适应性较
郴县茶树良种繁殖示范场,是1986年12月由农业部、湖南省农业厅、郴州行署、郴县人民政府联合投资建成的,1993年10月竣工验收、项目内容和质量完全合格。该场的建成为我省茶
The p53 tumor suppressor is a sequence-specific transcription factor that undergoes an abundance of post-translational modifications for its regulation and acti
The guanine-nucleotide exchange factor (GEF)RalGPS1a activates small GTPase Ral proteins such as RalA and RalB by stimulating the exchange of Ral bound GDP to G
Diverse subtypes of voltage-gated sodium channels (VGSCs) have been found throughout tissues of the brain,muscles and the heart.Neurotoxins extracted from the v
Gram-negative Enterobacteriaceae with resistance to carbapenem conferred by New Delhi metallo-β-lactamase 1 (NDM-1) are a type of newly discovered antibiotic-r
After years of development,biosensors based on imaging ellipsometry and biosensors based on total inteal reflection imaging ellipsometry have been successfully
Hepatocyte growth factor-regulated tyrosine kinase substrate (Hrs) is a key component of the endosomal sorting complexes required for transport and has been dem
Murine gammaherpesvirus 68 (MHV-68),a member of the gammaherpesvirus family,replicates robustly in permissive cell lines and is able to infect laboratory mice.M