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Myelin maintenance: axonal support required

Nature Neuroscience volume 13, pages 275–277 (2010)Cite this article

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Axonal integrity depends on an intact myelin sheath, but the role of the axon in myelin maintenance is more mysterious. A new study reports that preservation of the myelin sheath requires neuronal expression of the enigmatic prion protein.

The nervous system is a collection of highly specialized cells that carry out their individual functions through intricate and intimate interactions with surrounding cells. Until recently, however, most disorders of the nervous system were thought to result from primary defects in the affected cells (cell-autonomous abnormalities). For example, the loss of motor neurons in amyotrophic lateral sclerosis was generally thought to result from a defect in motor neurons and the loss of the specific neuronal populations that characterizes Huntington's disease, Parkinson's disease and cerebellar ataxia were thought, in each case, to be the results of cell-autonomous defects. Recently, however, it has become increasingly clear that the so-called support cells of the nervous system, astrocytes, oligodendrocytes and microglial cells, contribute to disease pathogenesis in these and other neurodegenerative disorders1. In addition, we have learned that abnormalities of the myelin sheath, which is produced by oligodendrocytes in the CNS and Schwann cells in the peripheral nervous system (PNS), can lead to substantial axonal degeneration2. Proving that turnabout is fair play, a study in this issue of Nature Neuroscience describes an adult-onset demyelinating disorder that originates not from an abnormality in Schwann cells, but from a defect in a neuronally expressed protein3. Perhaps even more surprising is the demonstration that the culprit axonal protein is the cellular prion protein PrP, which is the target of transmissible neurodegenerative disorders of the CNS.

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Figure 1: Myelin maintenance by PrPc.

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  1. Department of Neurology, The author is at the Jack Miller Center for Peripheral Neuropathy, The University of Chicago, Chicago, Illinois, USA.,

    Brian Popko

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  1. Brian Popko
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Correspondence to Brian Popko.

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Popko, B. Myelin maintenance: axonal support required. Nat Neurosci 13, 275–277 (2010). https://doi.org/10.1038/nn0310-275

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