Research Article Open Access
Volume 2 | Issue 2 | DOI: https://doi.org/10.33696/diabetes.1.018

Neuromotor Impairment, Hearing Loss and Blindness in a Preclinical Mouse Model of Charcot Marie-Tooth Disorder

  • 1In vivex, www.invivex.com, 177b avenue Louis Lumière, 34400 Lunel, France
  • 2COMET, 80, rue Augustin Fliche, 34091 Montpellier, France
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Corresponding Author

Sergio Gonzalez-Gonzalez, sergiogonzalez@invivex.com

Received Date: June 05, 2020

Accepted Date: June 18, 2020


Schwann cells produce myelin sheath around peripheral nerve axons. Myelination is critical for rapid propagation of action potentials, as illustrated by the large number of acquired and hereditary peripheral neuropathies, such as diabetic neuropathy or Charcot-Marie-Tooth (CMT) diseases, that are commonly associated with a process of demyelination. CMT is a hereditary motor and sensory disorder of the peripheral nervous system affecting about one in 2,500 people, and the pathomechanism of the disease remains poorly studied. Here, we described the demyelinating phenotype, hearing impairment and blindness observed in the CMT1A mouse model C3-PMP22 at 6 months old. We observed a decrease of sciatic nerve action potential velocity, neuromuscular disorders, sciatic nerve and auditory nerve demyelination, hearing loss and decrease visual acuity in CMT1A mice at 6 months old compared to control mice. Our results confirm that these mice represent a robust and validated model to study the peripheral neuropathy induced by CMT disorder allowing to determine the efficacy of new pharmacological candidates targeting demyelinating diseases such as CMT1A disorder.

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