Amyotrophic Lateral Sclerosis  – TDP-43 Transgenic Mouse Model

Target the pathological hallmark of Frontotemporal Dementia (FTD) and almost all Amyotrophic lateral sclerosis (ALS) patients using the TDP-43 Transgenic Mouse Model

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TDP-43 Transgenic Mouse Model Key Characteristics

TAR DNA binding protein (TDP-43) encoded by the TARDBP gene, is a major protein involved in the pathogenesis of Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Currently, several ALS mouse models that mimic the progressive loss of motoneurons in the motor cortex, brain stem, and spinal cord are available to test the efficacy of novel ALS therapies. Transgenic TDP-43 mouse models overexpress the mutant form of human TDP-43, leading to motoneuron degeneration and paralysis. 

As part of its specialized neurology contract research services, InnoSer performs efficacy studies using transgenic TDP-43 mouse models, including the TDP-43(WT) and TDP-43(Q331K) mouse models of ALS. Available functional readouts in the TDP-43 mouse models include motor function testing, as well as sciatic nerve conduction electrophysiology (nerve conduction velocity [NCV], and compound muscle action potential [CMAP]). 

✓ Available with and without disease-associated mutations.

✓  Abnormal accumulation of TDP-43.

✓  Motor deficits.

✓  Neuroinflammation. 

✓  Neurodegeneration.

European based preclinical CRO offering Infantile Epileptic Encaphalopathy Stxbp1 mouse models for drug development

As part of InnoSer’s neurology ALS mouse model portfolio, we also offer efficacy studies in mouse models with TDP-43 pathology, including seeding TDP-43 mouse models using recombinant and/or patient-derived seeds, as well as a SOD1-G93A transgenic ALS mouse model. However, as each model is unique, modelling distinct pathophysiological of ALS, we recommend you discuss the most appropriate model with our neurology study directors. 

InnoSer’s neurology expert team possesses relevant experience in working with multiple therapy types, including small molecules, peptides, enzymes, oligonucleotides, gene therapy (viral vectors, e.g., AAVs), and immunotherapies (antibody/vaccine immunotherapies). 

Your ALS Research Starts Here.

View study timelines, recommended readouts, and example data featuring behavioral across different ALS mouse models.

ALS sample data leaflet download preclinical mouse models of ALS

TDP-43 Transgenic Mouse Model Sample Data

TDP-43 Transgenic Mouse Models Readouts

Key Behavioral Readouts in the TDP-43 Transgenic Mouse Model

Biological Readouts

Test the efficacy of your treatments with the following biological readouts: 
  

    The People Behind Your Research

    Thomas Vogels, PhD Neurology study director InnoSer

    Thomas Vogels, PhD, In Vivo Neurology Study Director

    Leads an expert team of scientists with vast experience in our Neurology models to help you choose the right model and guide your optimal study design. We provide the solution to accelerating your drug development.

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