TDP-43 Transgenic Mouse Models

Target the pathological hallmark of frontotemporal dementia (FTD) and almost all Amyotrophic lateral sclerosis patients with our TDP-43 proteinopathy models

Home » Neurology Research Models and Services  » ALS models and services » ALS models – TDP-43 Transgenic Mouse Models
Amyotrophic lateral sclerosis (ALS) exists on a continuum with frontotemporal dementia (FTD), which also affects the frontal and temporal lobes. Several ALS mouse models mimic the progressive loss of motoneurons in the motor cortex, brain stem, and spinal cord. 

Mutations in the gene encoding TAR DNA-binding protein 43 (TDP-43) are associated with familial ALS, and virtually all sporadic ALS cases have TDP-43 inclusions. TDP-43 transgenic models overexpress the mutant form of human TDP-43, leading to motoneuron degeneration and paralysis.

InnoSer offers fee-for-service transgenic TDP-43 models, including the TDP-43WT, TDP-43Q331K and TDP-43A315T models. Mutant TDP-43 mouse models recapitulate the TDP-43 aggregates found in ALS and FTD.  

Take advantage of InnoSer’s preclinical research expertise, flexibility, and collaborative approach for your research. We support our clients in identifying new drugs or applications, characterizing their pharmacological properties, and conducting safety and efficacy testing with state-of-the-art readout capabilities and histopathological analysis. 

TDP-43 Transgenic Mouse Model key characteristics:

  • Available with and without disease-associated mutations.
  • Abnormal accumulation of TDP-43.
  • Motor deficits.
  • Neuroinflammation.
  • Neurodegeneration.

Find the right model for you.

Compare our model capabilities and discover which of our neurology platforms suits your research needs

Belgian based preclinical neurology CRO mouse models

Key readouts

Test the efficacy of your treatments in the following battery of behavioural tests: 

  • Spontaneous Behavior (PhenoTyper™) 
  • Neurological scoring 
  • Rotarod 
  • Balance beam  
  • CatWalk 
  • Grip strength 
  • Wire hanging 
  • Nest building
Test the efficacy of your treatments with the following biological readouts:

  • MSD (e.g. cytokines in brain/spinal cord, plasma NF-L) 
  • Immunohistochemistry (TDP-43 accumulation, neuroinflammation, neurodegeneration) 
  • Immunofluorescence and FISH

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AAALAC Accreditation

InnoSer has earned the AAALAC accreditation, demonstrating our commitment to responsible animal care and use. AAALAC International is a nonprofit organization that promotes the humane treatment of animals in science through voluntary accreditation and assessment programs. Our accreditation is valid for three years, incl. 2023. Read more about the AAALAC accreditation programme here.

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Animal Welfare

The 3Rs impact everything from policy and regulatory change to the development and uptake of new technologies and approaches. This is why Innoser has ongoing commitment and monitoring of these processes. The steps we practice maximize our ability to replace, reduce and refine animal involvement and facilitate our commitment to these principles when it comes to research and drug development.

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