Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.

doi:10.1074/jbc.M113.515940

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Article

Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.

The Journal of biological chemistry - 11 Apr 2014

Stribl Carola, Samara Aladin, Trümbach Dietrich, Peis Regina, Neumann Manuela, Fuchs Helmut, Gailus-Durner Valerie, Hrabě de Angelis Martin, Rathkolb Birgit, Wolf Eckhard, Beckers Johannes, Horsch Marion, Neff Frauke, Kremmer Elisabeth, Koob Sebastian, Reichert Andreas S, Hans Wolfgang, Rozman Jan, Klingenspor Martin, Aichler Michaela, Walch Axel Karl, Becker Lore, Klopstock Thomas, Glasl Lisa, Hölter Sabine M, Wurst Wolfgang, Floss Thomas

Abstract excerpt

The majority of amyotrophic lateral sclerosis (ALS) cases as well as many patients suffering from frontotemporal lobar dementia (FTLD) with ubiquitinated inclusion bodies show TDP-43 pathology, the protein encoded by the TAR DNA-binding protein (Tardbp) gene. We used recombinase-mediated cassette exchange to introduce an ALS patient cDNA into the mouse Tdp-43 locus. Expression levels of human A315T TDP-43 protein...

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PMID: 24515116
DOI: 10.1074/jbc.M113.515940
PMCID: PMC4036193

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

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