title: Changes in the Expression of FUS/TLS in Spinal Cords of SOD1 G93A Transgenic Mice and Correlation with Motor-Neuron Degeneration.
abstract: In order to searching the possible pathogenesis of amyotrophic lateral sclerosis (ALS), we examined the expression and distribution of FUS/TLS protein in the different anatomic regions, segments and neural cells of adult spinal cord at the different stages of the SOD1 wild-type and G93A transgenic mice using the fluorescent immunohistochemistry. Result revealed that, in the SOD1 wild-type mice, the FUS/TLS expression almost wasn't detected. However, in the SOD1 G93A mice, the FUS/TLS expression in the white matter was significantly more than that in the gray matter. In the white matter, the FUS/TLS expression in the anterior funiculus was more than that in the lateral funiculus more than that in the posterior funiculus. In the gray matter, the FUS/TLS expression in the ventral horn was more than that surrounding the central canal more than that in the dorsal horn. The FUS/TLS expression in the thoracic segment was more than that in the cervical segment more than that in the lumbar segment. Almost all FUS/TLS expressed in the nuclear of the GFAP positive cell at the onset stage, but it expressed in both the nuclear and the cytoplasm of the GFAP positive cell at the progression stage, almost didn't detected FUS/TLS expression in the NeuN and Oligo positive cells. The FUS/TLS expression was positively correlated with the neuron death. Our data suggested that the expressive increase and mislocalization of FUS/TLS in the astrocyte cell might cause the motor neuron degenerative death in the SOD1 G93A transgenic mice.
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Jiao
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Yi
authors.initials: Y
authors.lastname: Lu
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Huiting
authors.initials: H
authors.lastname: Liang
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Chunyan
authors.initials: C
authors.lastname: Tang
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Zhu
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.; Department of Biochemistry and Molecular Biology, College of Basic Medical Science, Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Neurology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi, China.
authors.forename: Renshi
authors.initials: R
authors.lastname: Xu
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000378:metabolism; D000818:Animals; D005260:Female; D007150:Immunohistochemistry; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D016472:Motor Neuron Disease / Q000235:genetics / Q000378:metabolism*; D034702:RNA-Binding Protein FUS / Q000235:genetics / Q000378:metabolism*; D013116:Spinal Cord / Q000378:metabolism*; D013482:Superoxide Dismutase / Q000235:genetics / Q000378:metabolism*
chemical_list: D034702:RNA-Binding Protein FUS; C109415:SOD1 G93A protein; D013482:Superoxide Dismutase
keywords: FUS/TLS; amyotrophic lateral sclerosis; astrocyte cell; pathogenesis.; spinal cord