Neuropathies & demyelinating diseases
A correct production and maintenance of myelin sheath is essential to the proper function of the peripheral nervous system (PNS). Resembling insulation of an electrical wire, the myelin sheath enables electrical signals to be rapidly and correctly conducted along the nerve fiber.
However, hereditary neuropathies are among the most common inherited neurological diseases (Charcot-Marie-Tooth disease, CMT). They typically affect myelinated peripheral axons and can be caused by defects in neurons and/or Schawnn cells. In demyelinating neuropathies (CMT1), the deleterious effects originate primarily in myelinating Schwann cells. In axonal neuropathies, neurons are initially affected (CMT2). Pathological hallmarks are a reduction of nerve conduction, alteration of axonal transport and a progressive axon loss leading to neurogenic muscle atrophy or sensory deficits.
Neuron Experts has developed an in vitro model of sensory neurons / Schwann cells co-cultures where both cell types originating from the same individual are cultured together in monolayer with the possibility to keep them in a serum free culture medium for 2-3months.
Primary culture of sensory neurons (Picture 1); Schwann cells; myelinating co-culture of Schwann cell and DRG sensory neurons (Picture 2).
Models of Intoxication:
- Anti-mitotic injuries (taxol, cisplatin, vincristine)
- TNFα injuries
- Induced demyelination by toxic agents
- Schwann cell survival and proliferation
- Differentiation and maturation of Schwann cells
- Loss of neurite
- Neuronal death / apoptosis evaluation
- Myelination of sensory neurons by Schwann cells ; kinetic of myelination
- Myelination markers expression