id	author	title	date	pages	extension	mime	words	sentences	flesch	summary	cache	txt
work_p3xvzfb5qnev5mxvfcqon4ggzq	Qihua Chen	Temporal Expression of Mutant TDP-43 Correlates with Early Amyotrophic Lateral Sclerosis Phenotype and Motor Weakness	2018	7	.pdf	application/pdf	3881	456	68	Temporal Expression of Mutant TDP-43 Correlates with Early Amyotrophic Lateral Sclerosis Phenotype and Motor Weakness Method: Transgenic rats were established that express mutant human TDP-43 (M337V substitution) in neurons, then a Tet-off system was used to regulate its expression. Keywords: Amyotrophic Lateral Sclerosis (ALS), TAR DNA-binding protein 43, motor neurons, transgenic rats, CAG, Tetresponsive transactivator. function in ALS rats expressing the causative gene in neurons and muscles, indicating that the motor neuron degeneration is partially reversible [10]. double transgenic rats were deprived of Dox to activate mutant TDP gene. To examine whether mutant TDP-43 expressed in neurons could induce ALS-like phenotypes, we chose the regulatory promoter of CAG gene to control the activation of of CAG-tTA/TRE-TDP-43M337V double transgenic rats suffered from progressive weakness after Dox (Fig. 1A, B). Mutant human TDP-43 expressed on 30 days causes shorter onset and induces more severe paralysis in transgenic rats.	./cache/work_p3xvzfb5qnev5mxvfcqon4ggzq.pdf	./txt/work_p3xvzfb5qnev5mxvfcqon4ggzq.txt