Peripheral Demyelinating Diseases: From Biology to Translational Medicine

Khidhir Kamil, Muhammad Dain Yazid, Ruszymah Bt Hj Idrus, Srijit Das, Jaya Kumar, Khidhir Kamil, Muhammad Dain Yazid, Ruszymah Bt Hj Idrus, Srijit Das, Jaya Kumar

Abstract

Demyelinating diseases represent a spectrum of disorders that impose significant burden on global economy and society. Generally, the prognosis of these diseases is poor and there is no available cure. In recent decades, research has shed some light on the biology and physiology of Schwann cells and its neuroprotective effects in the peripheral nervous system (PNS). Insults to the PNS by various infectious agents, genetic predisposition and immune-related mechanisms jeopardize Schwann cell functions and cause demyelination. To date, there are no effective and reliable biomarkers for PNS-related diseases. Here, we aim to review the following: pathogenesis of various types of peripheral demyelinating diseases such as Guillain-Barre syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Anti-Myelin Associated Glycoprotein Neuropathy, POEMS syndrome, and Charcot-Marie-Tooth disease; emerging novel biomarkers for peripheral demyelinating diseases, and Schwann cell associated markers for demyelination.

Keywords: Charcot-Marie-Tooth disease; Guillain-Barre syndrome; POEMS syndrome; anti-MAG neuropathy; biomarker; chronic inflammatory demyelinating polyradiculoneuropathy; peripheral demyelinating disease; schwann cell.

Figures

Figure 1
Figure 1
Pathogenesis of Guillain-Barre syndrome (GBS) and Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Figure 2
Figure 2
Demyelination and released of myelin associated protein. PMP22, peripheral myelin protein 22; P0, myelin protein zero; MBP, myelin basic protein; P2, myelin protein 2.
Figure 3
Figure 3
(A) Structure of myelin sheath of Schwann cell around axon under electron microscope. (B) Compact myelin that consist of major dense line and intraperiod line. NCM, non-compact myelin; CM, compact myelin; SC, Schwann cell; MDL, major dense line; IPL, intraperiod line; P0, myelin protein zero; MBP, myelin basic protein; PMP22, peripheral myelin protein 22.
Figure 4
Figure 4
Anatomical compartment of the node of Ranvier and myelin.

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