New evidence for secondary axonal degeneration in demyelinating neuropathies
| Autor: | Taylor S. Bopp, Ahmet Hoke, Anna E. Johnson, Kathryn R. Moss |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Schwann cell Chronic inflammatory demyelinating polyneuropathy Article 03 medical and health sciences Myelin Polyneuropathies 0302 clinical medicine Charcot-Marie-Tooth Disease medicine Animals Humans Axon Arthrogryposis Guillain-Barre syndrome business.industry General Neuroscience medicine.disease Genetic translation Axons 030104 developmental biology Peripheral neuropathy medicine.anatomical_structure nervous system Peripheral nervous system Nerve Degeneration Schwann Cells business Hereditary Sensory and Motor Neuropathy Neuroscience 030217 neurology & neurosurgery Demyelinating Diseases |
| Zdroj: | Neurosci Lett |
| ISSN: | 1872-7972 |
| Popis: | Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will eventually ensheath them. Myelin sheaths have evolved out of necessity to maintain rapid impulse propagation while accounting for body space constraints. However, myelinating SCs perform additional critical functions that are required to preserve axonal integrity including mitigating energy consumption by establishing the nodal architecture, regulating axon caliber by organizing axonal cytoskeleton networks, providing trophic and potentially metabolic support, possibly supplying genetic translation materials and protecting axons from toxic insults. The intermediate steps between the loss of these functions and the initiation of axon degeneration are unknown but the importance of these processes provides insightful clues. Prevalent demyelinating diseases of the PNS include the inherited neuropathies Charcot-Marie-Tooth Disease, Type 1 (CMT1) and Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) and the inflammatory diseases Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Secondary axon degeneration is a common feature of demyelinating neuropathies and this process is often correlated with clinical deficits and long-lasting disability in patients. There is abundant electrophysiological and histological evidence for secondary axon degeneration in patients and rodent models of PNS demyelinating diseases. Fully understanding the involvement of secondary axon degeneration in these diseases is essential for expanding our knowledge of disease pathogenesis and prognosis, which will be essential for developing novel therapeutic strategies. |
| Databáze: | OpenAIRE |
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