Publication: Distal axonopathy in peripheral nerves of PMP22-mutant mice.
Distal axonopathy in peripheral nerves of PMP22-mutant mice.
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Sancho, S., Magyar, J. P., Aguzzi, A., & Suter, U. (1999). Distal axonopathy in peripheral nerves of PMP22-mutant mice. Brain : A Journal of Neurology, 122(8), 1563–1577. https://doi.org/10.1093/brain/122.8.1563
Abstract
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Abstract
A partial duplication of chromosome 17 is associated with Charcot-Marie-Tooth disease type 1A (CMT1A), a demyelinating peripheral neuropathy that causes progressive distal muscle atrophy and sensory impairment. Trisomic expression of peripheral myelin protein 22 (PMP22) whose gene is contained within the duplicated region is considered to be responsible for the disease. By using recombinant gene technology in rodents, we had demonstrated previously that PMP22 is sensitive to gene dosage. Homozygous PMP22 knockout (PMP22(0/0)) mice and
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- Sancho, S
- Magyar, J P
- Suter, Ueli
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Sancho, S., Magyar, J. P., Aguzzi, A., & Suter, U. (1999). Distal axonopathy in peripheral nerves of PMP22-mutant mice. Brain : A Journal of Neurology, 122(8), 1563–1577. https://doi.org/10.1093/brain/122.8.1563
