Charcot Marie Tooth Disease Type 1A
(C3-PMP22)
Mutations in the PMP22 gene cause several forms of neuropathies such as Charcot-Marie-Tooth Disease Type 1A
Take advantage of InnoSer’s 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.
C3-PMP22 model key characteristics:
- The C3-PMP22 mouse model reverts back to the original C22-PMP22 model on its own after being bred with C57BL/6J mice.
- Model contains 3 to 4 copies of the human PMP22 gene.
- In comparison to the original C22-PMP22 model, the C3-PMP22 model shows a milder disease phenotype, better resembling the human disease course of CMT1A.
- Model is extensively characterised (findings published together with Michailidou et al. 2023).
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Key readouts
Test the efficacy of your treatments in the following battery of behavioural tests:
- Spontaneous behaviour (automatic home-cages)
- CatWalk: Automated Gait Analysis
- Balance Beam
- Grip Strength Test
Neuromuscular function
- Compound muscle action potential (CMAP)
- Sensory nerve conduction velocity (SNCV)
- Sciatic nerve histology
C3-PMP22 mice have reduced motor function, as indicated by a higher number of slips on the balance beam
The Balance Beam sensorimotor coordination test scores the ability of mice to traverse a stationary horizontal rod. The coordination of the mouse is measured by the latency to cross the beam and the number of foot slips made.
Related rare disease model options
Infantile Epileptic Encephalopathy
Fragile X Syndrome
Vanishing White Matter
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.
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.
References
- Michailidou I, Vreijling J, Rumpf M, Loos M, Koopmans B, Vlek N, Straat N, Agaser C, Kuipers TB, Mei H, Baas F. The systemic inhibition of the terminal complement system reduces neuroinflammation but does not improve motor function in mouse models of CMT1A with overexpressed PMP22. Current Research in Neurobiology. 2023 Jan 1;4:100077.
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