TDP-43 Models of Amyotrophic Lateral Sclerosis (ALS)

We offer preclinical studies with a regulatable human TDP-43 mouse model. This model is often used as a model of amyotrophic lateral sclerosis (ALS) and other TDP-43 proteinopathies, such as frontotemporal dementia (FTD) and other forms of frontotemporal lobar degeneration (FTLD).

The TDP-43 model involves cytoplasmic TDP-43 aggregates that are regulatable and reversible via dox diet. This model demonstrates brain neurodegeneration, spinal motor neuron loss, and muscle denervation, resulting in a progressive, neurodegenerative phenotype.  This neurodegeneration model also shows highly reproducible motor deficits, brain atrophy by MRI, elevation of neurofilament light (NfL) in plasma & CSF, and astrogliosis.  We are able to provide this model either as a rapidly progressing model or as a slower progressing model.  


We can also inject these mice with human brain or spinal cord extracts to induce seeding and spreading of TDP-43 pathology. 

This rodent model is used in preclinical efficacy assessments of therapeutics related to

  • Misfolded TDP-43 aggregates
  • Neuroprotection
  • Neuroinflammation
  • Mitochondrial dysfunction

At Biospective, we offer preclinical studies with our well-characterized and validated in-house models, as well as with commercially-available and sponsor-provided models. Our end-to-end preclinical studies include breeding, housing, dosing, in vivo imaging (MRI & PET), behavior and functional assessments, and immunohistochemistry (IHC).