Document Detail


Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis.
MedLine Citation:
PMID:  23317354     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Mohammad A Esmaeili; Marzieh Panahi; Shilpi Yadav; Leah Hennings; Mahmoud Kiaei
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  International journal of experimental pathology     Volume:  94     ISSN:  1365-2613     ISO Abbreviation:  Int J Exp Pathol     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-01-15     Completed Date:  2013-03-05     Revised Date:  2014-02-04    
Medline Journal Info:
Nlm Unique ID:  9014042     Medline TA:  Int J Exp Pathol     Country:  England    
Other Details:
Languages:  eng     Pagination:  56-64     Citation Subset:  IM    
Copyright Information:
© 2012 The Authors. International Journal of Experimental Pathology © 2012 International Journal of Experimental Pathology.
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MeSH Terms
Descriptor/Qualifier:
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:
5P20RR020146-09/RR/NCRR NIH HHS; 8 P20 GM103425-09/GM/NIGMS NIH HHS
Chemical
Reg. No./Substance:
0/DNA-Binding Proteins; 0/protein TDP-43
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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