| Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis. | |
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MedLine Citation:
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PMID: 23317354 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Abnormal distribution, modification and aggregation of transactivation response DNA-binding protein 43 (TDP-43) are the hallmarks of multiple neurodegenerative diseases, especially frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS). Transgenic mouse lines overexpressing wild-type or mutant TDP-43 exhibit ALS-like symptom, motor abnormalities and early paralysis followed by death. Reports on lifespan and phenotypic behaviour in Prp-TDP-43 (A315T) vary, and these animals are not fully characterized. Although it has been proposed that the approximate 20% loss of motor neurons at end stage is responsible for the severe weakness and death in TDP-43 mice, this degree of neurologic damage appears insufficient to cause death. Hence we studied these mice to further characterize and determine the reason for the death. Our characterization of TDP-43 transgenic mice showed that these mice develop ALS-like symptoms that later become compounded by gastrointestinal (GI) complications that resulted in death. This is the first report of a set of pathological evidence in the GI track that is strong indicator for the cause of death of Prp-hTDP-43 (A315T) transgenic mice. |
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Authors:
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Mohammad A Esmaeili; Marzieh Panahi; Shilpi Yadav; Leah Hennings; Mahmoud Kiaei |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: International journal of experimental pathology Volume: 94 ISSN: 1365-2613 ISO Abbreviation: Int J Exp Pathol Publication Date: 2013 Feb |
Date Detail:
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Created Date: 2013-01-15 Completed Date: 2013-03-05 Revised Date: 2013-04-16 |
Medline Journal Info:
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Nlm Unique ID: 9014042 Medline TA: Int J Exp Pathol Country: England |
Other Details:
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Languages: eng Pagination: 56-64 Citation Subset: IM |
Copyright Information:
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© 2012 The Authors. International Journal of Experimental Pathology © 2012 International Journal of Experimental Pathology. |
Affiliation:
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Department of Neurobiology and Developmental Sciences, Center for Translational Neuroscience, University of Arkansas for Medical Sciences, Little Rock, AR, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Age Factors Amyotrophic Lateral Sclerosis / genetics, metabolism*, pathology, physiopathology Animals Behavior, Animal Cecum / metabolism, pathology DNA-Binding Proteins / genetics, metabolism* Disease Progression Female Gastrointestinal Diseases / genetics, metabolism*, pathology, physiopathology Genetic Predisposition to Disease Humans Ileum / metabolism, pathology Intestines / metabolism*, pathology Male Mice Mice, Inbred C57BL Mice, Transgenic Motor Activity Phenotype Spinal Cord / metabolism*, pathology |
| Grant Support | |
ID/Acronym/Agency:
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5P20RR020146-09/RR/NCRR NIH HHS; 8 P20 GM103425-09/GM/NIGMS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/DNA-Binding Proteins; 0/protein TDP-43 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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