HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice

被引：52

作者：

Guo, Yansu ^{[1
,2
,3
]}

Wang, Qian ^{[1
]}

Zhang, Kunxi ^{[1
]}

An, Ting ^{[1
]}

Shi, Pengxiao ^{[1
]}

Li, Zhongyao ^{[1
,2
]}

Duan, Weisong ^{[1
,2
,3
]}

Li, Chunyan ^{[1
,2
,3
]}

机构：

[1] Hebei Med Univ, Hosp 2, Dept Neurol, Shijiazhuang 050000, Hebei Province, Peoples R China

[2] Key Lab Hebei Neurol, Shijiazhuang 050000, Hebei Province, Peoples R China

[3] Inst Cardiocerebrovasc Dis, Shijiazhuang 050000, Hebei Province, Peoples R China

来源：

BRAIN RESEARCH | 2012年 / 1460卷

基金：

中国国家自然科学基金;

关键词：

Amyotrophic lateral sclerosis; TDP-43 A315T transgenic mice; Autophagy; Antioxidant enzyme; Glia; AMYOTROPHIC-LATERAL-SCLEROSIS; FRONTOTEMPORAL LOBAR DEGENERATION; PARKINSONISM-DEMENTIA COMPLEX; BINDING PROTEIN 43; MUTANT SOD1 GENE; TAR-DNA-BINDING; PATHOLOGICAL TDP-43; SPINAL-CORD; DISEASE; AUTOPHAGY;

D O I：

10.1016/j.brainres.2012.04.003

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

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.

引用

页码：88 / 95

页数：8

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