title: HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice.
abstract: TAR DNA-binding protein 43 (TDP-43) has been found to be related to the pathogenesis of amyotrophic lateral sclerosis (ALS). TDP-43 A315T transgenic mice develop degeneration of specific motor neurons, and accumulation of ubiquitinated proteins has been observed in the pyramidal cells of motor cortex of these mice. In this study, we found stress-responsive HO-1 induction and no autophagic alteration in motor cortex of TDP-43 A315T transgenic mice. Glial activation, especially astrocytic proliferation, occurred in cortical layer 5 and sub-meningeal region. Interestingly, we noticed that progressively thinned colon, swollen small intestine and reduced food intake, rather than severe muscle weakness, contributed to the death of TDP-43 A315T transgenic mice. Increased TDP-43 accumulation in the myenteric nerve plexus and increased thickness of muscular layer of colon were related to the intestinal dysfunction.
authors.affiliation: Department of Neurology, The Second Hospital of Hebei Medical University, Hebei 050000, China.
authors.forename: Yansu
authors.initials: Y
authors.lastname: Guo
authors.forename: Qian
authors.initials: Q
authors.lastname: Wang
authors.forename: Kunxi
authors.initials: K
authors.lastname: Zhang
authors.forename: Ting
authors.initials: T
authors.lastname: An
authors.forename: Pengxiao
authors.initials: P
authors.lastname: Shi
authors.forename: Zhongyao
authors.initials: Z
authors.lastname: Li
authors.forename: Weisong
authors.initials: W
authors.lastname: Duan
authors.forename: Chunyan
authors.initials: C
authors.lastname: Li
mesh_terms: D000818:Animals; D001343:Autophagy / Q000235:genetics; D004268:DNA-Binding Proteins / Q000096:biosynthesis / Q000235:genetics*; D051547:Heme Oxygenase-1 / Q000096:biosynthesis / Q000235:genetics*; D006801:Humans; D007410:Intestinal Diseases / Q000201:enzymology / Q000503:physiopathology*; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009044:Motor Cortex / Q000201:enzymology* / Q000473:pathology / Q000503:physiopathology; D057177:TDP-43 Proteinopathies / Q000201:enzymology / Q000235:genetics / Q000378:metabolism*; D015533:Transcriptional Activation / Q000235:genetics; D015854:Up-Regulation / Q000235:genetics
chemical_list: D004268:DNA-Binding Proteins; D051547:Heme Oxygenase-1