Abstract
Demyelinating pathologies comprise of a variety of conditions where either central or peripheral myelin is attacked, resulting in white matter lesions and neurodegeneration. Myelinated axons are organized into molecularly distinct domains, and this segregation is crucial for their proper function. These defined domains are differentially affected at the different stages of demyelination as well as at the lesion and perilesion sites. Among the main players in myelinated axon organization are proteins of the contactin (CNTN) group of the immunoglobulin superfamily (IgSF) of cell adhesion molecules, namely Contactin-1 and Contactin-2 (CNTN1, CNTN2). The two contactins perform their functions through intermolecular interactions, which are crucial for myelinated axon integrity and functionality. In this review, we focus on the implication of these two molecules as well as their interactors in demyelinating pathologies in humans. At first, we describe the organization and function of myelinated axons in the central (CNS) and the peripheral (PNS) nervous system, further analyzing the role of CNTN1 and CNTN2 as well as their interactors in myelination. In the last section, studies showing the correlation of the two contactins with demyelinating pathologies are reviewed, highlighting the importance of these recognition molecules in shaping the function of the nervous system in multiple ways.
Highlights
Laboratory of Neuroscience, Department of Basic Science, University of Crete Medical School and IMBB FORTH, Citation: Kalafatakis, I.; Savvaki, M.; Abstract: Demyelinating pathologies comprise of a variety of conditions where either central or peripheral myelin is attacked, resulting in white matter lesions and neurodegeneration
The Nav channels are distributed along the axon, in myelinated axons, they are clustered in the nodes [18,19]
In contrast to the peripheral nervous system (PNS), in the central nervous system (CNS), there are three complementary mechanisms participating in nodal assembly: extracellular matrix (ECM)-induced cell– adhesion molecule clustering, paranodal barrier formation and scaffolding molecules anchoring the nodal complex to the actin cytoskeleton [27]
Summary
Myelinated axons are characterized by exquisite architectural and functional specificity. They are endowed with the task of rapid signal propagation, which they effectively accomplish by segregating large adhesion protein complexes and ion channels along the axon, generating distinct functional and morphological domains, namely the node of Ranvier, paranode (PNJ), juxtaparanode (JXP) and internode (Figure 1) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16]
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