21408206

Source:http://linkedlifedata.com/resource/pubmed/id/21408206

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Predicate	Object
rdf:type	pubmed:Citation
lifeskim:mentions	umls-concept:C0002736, umls-concept:C0031437, umls-concept:C0751072, umls-concept:C1414860, umls-concept:C2350483
pubmed:issue	3
pubmed:dateCreated	2011-3-16
pubmed:abstractText	Fused in Sarcoma (FUS) proteinopathy is a feature of frontotemporal lobar dementia (FTLD), and mutation of the fus gene segregates with FTLD and amyotrophic lateral sclerosis (ALS). To study the consequences of mutation in the fus gene, we created transgenic rats expressing the human fus gene with or without mutation. Overexpression of a mutant (R521C substitution), but not normal, human FUS induced progressive paralysis resembling ALS. Mutant FUS transgenic rats developed progressive paralysis secondary to degeneration of motor axons and displayed a substantial loss of neurons in the cortex and hippocampus. This neuronal loss was accompanied by ubiquitin aggregation and glial reaction. While transgenic rats that overexpressed the wild-type human FUS were asymptomatic at young ages, they showed a deficit in spatial learning and memory and a significant loss of cortical and hippocampal neurons at advanced ages. These results suggest that mutant FUS is more toxic to neurons than normal FUS and that increased expression of normal FUS is sufficient to induce neuron death. Our FUS transgenic rats reproduced some phenotypes of ALS and FTLD and will provide a useful model for mechanistic studies of FUS-related diseases.
pubmed:grant	http://linkedlifedata.com/resource/pubmed/grant/NS072113, http://linkedlifedata.com/resource/pubmed/grant/NS072696, http://linkedlifedata.com/resource/pubmed/grant/R01 NS072113-02, http://linkedlifedata.com/resource/pubmed/grant/R21 NS072696-01, http://linkedlifedata.com/resource/pubmed/grant/R21 NS072696-02
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pubmed:language	eng
pubmed:journal	http://linkedlifedata.com/resource/pubmed/journal/101239074
pubmed:citationSubset	IM
pubmed:chemical	http://linkedlifedata.com/resource/pubmed/chemical/RNA-Binding Protein FUS, http://linkedlifedata.com/resource/pubmed/chemical/Ubiquitin
pubmed:status	MEDLINE
pubmed:month	Mar
pubmed:issn	1553-7404
pubmed:author	pubmed-author:ChenHanH, pubmed-author:HuangCaoC, pubmed-author:LiuYong-JianYJ, pubmed-author:TongJianbinJ, pubmed-author:WangDianD, pubmed-author:WeiXiaotaoX, pubmed-author:XiaXu-GangXG, pubmed-author:ZhouHongxiaH
pubmed:issnType	Electronic
pubmed:volume	7
pubmed:owner	NLM
pubmed:authorsComplete	Y
pubmed:pagination	e1002011
pubmed:dateRevised	2011-10-7
pubmed:meshHeading	pubmed-meshheading:21408206-Amyotrophic Lateral Sclerosis, pubmed-meshheading:21408206-Animals, pubmed-meshheading:21408206-Disease Models, Animal, pubmed-meshheading:21408206-Frontotemporal Dementia, pubmed-meshheading:21408206-Humans, pubmed-meshheading:21408206-Mutagenesis, Site-Directed, pubmed-meshheading:21408206-Mutation, pubmed-meshheading:21408206-Neuroglia, pubmed-meshheading:21408206-Neurons, pubmed-meshheading:21408206-RNA-Binding Protein FUS, pubmed-meshheading:21408206-Rats, pubmed-meshheading:21408206-Rats, Transgenic, pubmed-meshheading:21408206-Ubiquitin
pubmed:year	2011
pubmed:articleTitle	FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
pubmed:affiliation	Department of Pathology, Anatomy, and Cell Biology, Thomas Jefferson University, Philadelphia, Pennsylvania, United States of America.
pubmed:publicationType	Journal Article, Research Support, N.I.H., Extramural