TDP-43PrP transgenic mice express a full length wild type human TAR DNA binding protein (TARDBP or TDP-43) cDNA under control of the prion protein (Prnp) promoter. Hemizygotes are viable, fertile, and normal in size. TDP-43 is a ubiquinated protein localized to the nucleus of cells. Accumulations of TDP-43 are involved in the development of Amyotrophic lateral sclerosis (ALS). These mice express wildtype human TDP-43 primarily in the nuclei of neurons throughout much of the gray matter of the spinal cord and brain, including the hippocampus, striatum, brainstem and cortex. Homozygotes and hemizygous express TDP-43 in brain at 2.5 and 1.9-fold endogenous TDP-43 levels, respectively. Endogenous TDP-43 is down-regulated in response to transgene overexpression. Beginning at 14 days of age, homozygous TDP-43PrP are smaller than hemizygous littermates. By 21 days of age homozygotes develop body tremors, have difficulty walking, and develop an aberrant "swimming" gait. Between 1 and 2 months of age they become unable to right themselves. These also mice exhibit reactive gliosis, and axonal and myelin degeneration. These mice exhibit truncated TDP-43 and ubiquitin aggregates containing phosphorylated TDP-43. Hemizygous mice are indistinguishable from wildtype littermates. These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Lou Gehrig's Disease) and frontotemporal lobar degeneration with ubiquitin aggregates.

TDP-43PrP transgenic mice express a full length wild type human TARDBP cDNA under control of a Prnp promoter. These transgenic mice may be useful in studying neuromuscular and neurodegenerative disorders such as ALS (Lou Gehrig's Disease).

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