Journal
BRAIN RESEARCH
Volume 1460, Issue -, Pages 88-95Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.brainres.2012.04.003
Keywords
Amyotrophic lateral sclerosis; TDP-43 A315T transgenic mice; Autophagy; Antioxidant enzyme; Glia
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Funding
- National Natural Science Foundation of China [30900460, 81171210]
- Hebei Science and Technology Department [11966122D]
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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. (C) 2012 Elsevier B.V. All rights reserved.
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