New evidence for secondary axonal degeneration in demyelinating neuropathies. (23rd January 2021)

• Record Type: Journal Article
• Authors: Moss, Kathryn R.; Bopp, Taylor S.; Johnson, Anna E.; Höke, Ahmet
• Is Part Of: Neuroscience letters. Volume 744(2021)
• Publication Date: 2021-01-23
• Subjects: AIDP Acute Inflammatory Demyelinating Polyneuropathy -- ATP Adenosine Triphosphate -- CNS Central Nervous System -- CMT Charcot-Marie-Tooth Disease -- CMT Charcot-Marie-Tooth Disease, Type 1 -- CMT1A Charcot-Marie-Tooth Disease, Type 1A -- CMT1B Charcot-Marie-Tooth Disease, Type 1B -- CMT1C Charcot-Marie-Tooth Disease, Type 1C -- CMT1D Charcot-Marie-Tooth Disease, Type 1D -- CMT1E Charcot-Marie-Tooth Disease, Type 1E -- CMT1F Charcot-Marie-Tooth Disease, Type 1F -- CMT1X Charcot-Marie-Tooth Disease, Type 1X -- CMT2 Charcot-Marie-Tooth Disease, Type 2 -- CIDP Chronic Inflammatory Demyelinating Polyneuropathy -- iSCs Immature Schwann Cells -- CNTF Ciliary Neurotrophic Factor -- CMAP Compound Muscle Action Potential -- Cx3 Connexin 32 -- CNTN1 Contactin-1 -- CASPR Contactin Associated Protein-like 1 -- DADS Distal Acquired Demyelinating Symmetric Neuropathy -- EGR2 Early Growth Response 2 -- ER Endoplasmic Reticulum -- EAN Experimental Autoimmune Neuritis -- GJB Gap Junction Protein Beta 1 -- GBS Guillain-Barré Syndrome -- HNPP Hereditary Neuropathy with Liability to Pressure Palsies -- LITAF Lipopolysaccharide Induced TNF Factor -- MCV Motor Conduction Velocity -- MADSAM Multifocal Acquired Demyelinating Sensory and Motor Neuropathy -- MAG Myelin Associated Glycoprotein -- MPZ Myelin Protein Zero -- NCV Nerve Conduction Velocity -- NF Neurofascin -- NF-155 Neurofascin-155 -- NF-186 Neurofascin-186 -- NEFL Neurofilament Light -- NT-3 Neurotrophin-3 -- NAD+ Nicotinamide Adenine Dinucleotide -- PRX Periaxin -- PMP2 Peripheral Myelin Protein 2 -- PMP22 Peripheral Myelin Protein 22 -- PNS Peripheral Nervous System -- SC Schwann Cell -- SCPs Schwann Cell Precursors -- SCV Sensory Conduction Velocity -- SNAP Sensory Nerve Action Potential -- SARM1 Sterile Alpha and TIR Motif Containing 1 -- WD Wallerian Degeneration -- WLD Wallerian-like Degeneration; Acute inflammatory demyelinating polyneuropathy -- AIDP -- Charcot-Marie-Tooth disease -- Chronic inflammatory demyelinating polyneuropathy -- CIDP -- CMT -- CMT1A -- CMT1B -- CMT1C -- CMT1D -- CMT1E -- CMT1F -- CMT1X -- Cx32 -- Demyelination -- EGR2 -- GBS -- GJB1 -- Guillain-Barré syndrome -- Hereditary -- Neuropathy with liability to pressure palsies -- HNPP -- LITAF/SIMPLE -- MPZ -- Myelin -- NEFL -- Peripheral neuropathy -- PMP22 -- Secondary axon degeneration; Neurology -- Periodicals; Neurology -- Periodicals; Research -- Periodicals; Neurologie -- Périodiques; Neuroanatomie -- Périodiques; Neuropharmacologie -- Périodiques; Neurophysiologie -- Périodiques; Neurology; Periodicals; Electronic journals; 617.48
• DOI: 10.1016/j.neulet.2020.135595
• Languages: English
• ISSNs: 0304-3940

Abstract

Highlights: Summary of the role of myelin and myelinating Schwann cells. Review of the effects of myelination and demyelination on axon integrity and axon degeneration pathways. Review of secondary axon degeneration in demyelinating Charcot- Marie-Tooth disease. Review of axon degeneration in acquired demyelinating neuropathies, GBS and CIDP. Abstract: 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.