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Updated: Jul 5, 2025

Preparation and Immunostaining of Myelinating Organotypic Cerebellar Slice Cultures
Published on: March 20, 2019
Peripheral Nervous System (PNS) Myelin Diseases
Steven S Scherer1, John Svaren2
1Department of Neurology, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA sscherer@pennmedicine.upenn.edu.
Abstract:
This is a review of inherited and acquired causes of human demyelinating neuropathies and a subset of disorders that affect axon-Schwann cell interactions. Nearly all inherited demyelinating neuropathies are caused by mutations in genes that are expressed by myelinating Schwann cells, affecting diverse functions in a cell-autonomous manner. The most common acquired demyelinating neuropathies are Guillain-Barré syndrome and chronic, inflammatory demyelinating polyneuropathy, both of which are immune-mediated. An additional group of inherited and acquired disorders affect axon-Schwann cell interactions in the nodal region. Overall, these disorders affect the formation of myelin and its maintenance, with superimposed axonal loss that is clinically important.
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