These PrP-TDP43-IRES-EGFP line 19 transgenic mice (TDP-43 Tg19) have the mouse prion promoter directing expression of mouse TDP-43 cDNA as well as EGFP. The modest levels of TDP-43 overexpression in the central nervous system (CNS) result in diverse aspects of amyotrophic lateral sclerosis (ALS; Lou Gehrig&rsquo;s disease) including late-onset progressive motor dysfunction ending in paralysis in mid-life, spinal cord deficits (progressive oligodendrocyte degeneration, demyelination, astrogliosis, microgliosis, neuroinflammation), distal motor axon degeneration and neuromuscular denervation in the periphery and motor neuron loss in layer V cortex. No loss of lower motor neurons in the lumbar spinal cord was observed (despite complete paralysis of the hindlimbs). Homozygotes have TDP-43 expression increased ~30% in spinal cord and ~40% in frontal cortex as compared to non-transgenic mice. Homozygotes may begin to exhibit the defects described above by ~250 days old and develop paralysis by ~300-430 days old. These mice went through a hyperactive stage before developing weakness and paralysis. Hemizygotes have TDP-43 expression increased ~10% in spinal cord and ~20% in frontal cortex as compared to non-transgenic mice. Hemizygotes exhibit motor weakness after ~500 day old that progresses to paralysis a few months later. However, the phenotype is not fully penetrant up to 700 days of age. The PrP-TDP43-IRES-EGFP transgene is primarily expressed in neurons, astrocytes and oligodendrocytes in the cortex, but only in astrocytes and oligodendrocytes in the spinal cord. Transgene expression levels were substantially lower in heart, lung and kidney, and were undetectable in muscle, liver and spleen. The donating investigator reports that EGFP can only be detected by antibody staining. Of note, the donating investigator reports that while the transgene insertion site and copy number are not known to date (October 2020), the expression level and homozygous phenotype have been stable and consistent throughout their use of the mice. Importantly, the transgene is flanked with loxP sites. Although not specifically tested to date (October 2020), exposure to Cre recombinase is likely to delete the transgene.

PrP-TDP43-IRES-EGFP line 19 transgenic mice (TDP-43 Tg19) have modest levels of TDP-43 overexpression in the central nervous system that results in diverse aspects of amyotrophic lateral sclerosis (ALS; Lou Gehrig's disease). This modest/chronic elevation of TDP-43 may be useful for studying ALS and other neuromuscular conditions involving TDP-43 proteinopathy.

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