Genetics and cell biology

Biology of myelin

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Group Leader

Maurizio D'Antonio

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Myelin is a specialized structure that surrounds large axons and permits rapid conduction of signals. It is formed by oligodendrocytes in the central nervous system and Schwann cells in the peripheral nervous system. During development, these cells migrate with the axons that they will myelinate, and depend on those same axons for appropriate signals to survive and differentiate. Myelin-forming glia coordinately express a unique set of genes encoding myelin structural proteins, and enzymes that synthesize myelin lipids-this coordination is in large part transcriptionally-mediated. Mutations in these proteins, including myelin specific proteins such as P0 and PMP22, are associated in humans to hereditary neuropathies, collectively known as Charcot-Marie-Tooth (CMT) disease.

This unit is devoted to study the molecular mechanisms underlying these diseases, with the goal of developing effective therapeutic strategies.