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Understanding myelin biology in development and neurological disorders

Myelin is a lipid-rich cellular structure that surrounds axons. It is produced by special glial cells: the oligodendrocyte in the Central Nervous System (CNS) and the Schwann cell in the Peripheral Nervous System (PNS).  Myelin integrity is crucial as it enables fast nerve conduction, modulates nerve impulse conduction velocity, and promotes neuronal survival.

 

Abnormal myelin formation and maintenance, myelin breakdown and demyelination are common pathological features found in many CNS neurodegenerative disorders such as Multiple Sclerosis, Krabbe leukodystrophy and traumatic brain injury and PNS disorders including Charcot-Marie-Tooth disease, Guillain-Barré syndrome, diabetic and chemo-induced neuropathies.  These diseases are caused by various factors (inflammation, infection, metabolic changes and genetic mutations) that affect myelination, myelin maintenance and/or myelin repair. Understanding the molecular mechanisms that regulate these processes is important for developing therapeutic strategies to improve the prognosis of the patients.

 

We use mouse models and cell culture systems to decipher the intricate molecular and metabolic processes that are responsible for these myelin defects in human neuropathologies.

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