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2011 | 71 | S |

Tytuł artykułu

TDP-43 transgenic rat - animal model to study ALS and FTLD-U

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Języki publikacji

EN

Abstrakty

EN
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U) are characterized by mislocalisation and aggregation of predominantly nuclear TDP-43 protein. Our aim was to generate transgenic rats overexpressing wild type of human TDP-43 gene, fused with EGFP and under the control of synapsin promoter. Using lentiviral vectors as a tool for transgenesis, three founders carrying human TDP-43 gene were generated. The incorporation of transgene into genome was confirmed by specific PCR reaction and transgene copy number was determined by Real-Time quantitative PCR. Microscopic visualization and western blotting technique showed an abundant expression of GFP - fused TDP-43 protein in the brains of 3 months old animals. Notably, at this age no gross degeneration of neurons was detected. RotaRod test conducted on 5 months old transgenic rats showed no distinct motor deficits. At present we carry out behavioral analyses to verify whether animals exhibit cognitive impairments, which could possibly appear before any motor disorders. As neurodegeration occurs in ALS and FTLD-U patients in age-dependent manner, we plan to run further morphological and behavioral analyses on aging transgenic rats.

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-

Rocznik

Tom

71

Numer

S

Opis fizyczny

p.72

Twórcy

autor
  • Department of Molecular and Cellular Neurobiology, Nencki Institute of Experimental Biology PAS, Warsaw, Poland
autor
  • Laboratory of Cell Engineering, Nencki Institute of Experimental Biology PAS, Warsaw, Poland
autor
  • Department of Molecular and Cellular Neurobiology, Nencki Institute of Experimental Biology PAS, Warsaw, Poland

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