Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats

Mutation of Tar DNA‐binding protein 43 (TDP‐43) is linked to amyotrophic lateral sclerosis. Although astrocytes have important roles in neuron function and survival, their potential contribution to TDP‐43 pathogenesis is unclear. Here, we created novel lines of transgenic rats that express a mutant...

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Veröffentlicht in:	The EMBO journal 2013-07, Vol.32 (13), p.1917-1926
Hauptverfasser:	Tong, Jianbin, Huang, Cao, Bi, Fangfang, Wu, Qinxue, Huang, Bo, Liu, Xionghao, Li, Fang, Zhou, Hongxia, Xia, Xu-Gang
Format:	Artikel
Sprache:	eng
Schlagworte:	amyotrophic lateral sclerosis Amyotrophic Lateral Sclerosis - etiology Amyotrophic Lateral Sclerosis - metabolism Amyotrophic Lateral Sclerosis - pathology Animals astrocytes Astrocytes - metabolism Astrocytes - pathology Behavior, Animal Blotting, Western Cell Death Deoxyribonucleic acid Disease Models, Animal DNA DNA damage DNA-Binding Proteins - genetics DNA-Binding Proteins - metabolism EMBO24 EMBO27 Excitatory Amino Acid Transporter 1 - metabolism Excitatory Amino Acid Transporter 2 - metabolism Fluorescent Antibody Technique Gene expression Humans Immunoenzyme Techniques Lcn2 Lipocalin-2 Lipocalins - metabolism Mortality Motor Neurons - metabolism Motor Neurons - pathology Muscle Denervation Muscles Muscular Atrophy - etiology Muscular Atrophy - pathology Mutants Mutation Mutation - genetics Neurodegeneration Paralysis - etiology Paralysis - pathology Pathogenesis Rats Rats, Transgenic Spinal Cord - metabolism Spinal Cord - pathology TAR DNA-binding protein 43 Transgenic animals
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