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Role of connexin-based gap junction channels in communication of myelin sheath in schwann cells

dc.contributor.authorCisterna B.A.
dc.contributor.authorArroyo P.
dc.contributor.authorPuebla C.
dc.date.accessioned2020-09-02T22:14:54Z
dc.date.available2020-09-02T22:14:54Z
dc.date.issued2019
dc.identifier10.3389/fncel.2019.00069
dc.identifier.citation13, , 1-8
dc.identifier.issn16625102
dc.identifier.urihttps://hdl.handle.net/20.500.12728/4029
dc.descriptionPeripheral nerves have the capacity to conduct action potentials along great distances and quickly recover following damage which is mainly due to Schwann cells (SCs), the most abundant glial cells of the peripheral nervous system (PNS). SCs wrap around an axonal segment multiple times, forming a myelin sheath, allowing for a significant increase in action potential conduction by insulating the axons. Mature myelin consists of compact and non-compact (or cytoplasmic) myelin zones. Non-compact myelin is found in paranodal loops bordering the nodes of Ranvier, and in the inner and outermost cytoplasmic tongues and is the region in which Schmidt-Lanterman incisures (SLI; continuous spirals of overlapping cytoplasmic expansions within areas of compact myelin) are located. Using different technologies, it was shown that the layers of non-compact myelin could be connected to each other by gap junction channels (GJCs), formed by connexin 32 (Cx32), and their relative abundance allows for the transfer of ions and different small molecules. Likewise, Cx29 is expressed in the innermost layer of the myelin sheath. Here it does not form GJCs but colocalizes with Kv1, which implies that the SCs play an active role in the electrical condition in mammals. The critical role of GJCs in the functioning of myelinating SCs is evident in Charcot-Marie-Tooth disease (CMT), X-linked form 1 (CMTX1), which is caused by mutations in the gap junction protein beta 1 (GJB1) gene that codes for Cx32. Although the management of CMT symptoms is currently supportive, there is a recent method for targeted gene delivery to myelinating cells, which rescues the phenotype in KO-Cx32 mice, a model of CMTX1. In this mini-review article, we discuss the current knowledge on the role of Cxs in myelin-forming SCs and summarize recent discoveries that may become a real treatment possibility for patients with disorders such as CMT. © 2019 Cisterna, Arroyo and Puebla.
dc.language.isoen
dc.publisherFrontiers Media S.A.
dc.subjectCharcot-Marie-Tooth disease
dc.subjectCMTX1
dc.subjectConnexins
dc.subjectGap junction channels
dc.subjectMyelin sheath
dc.subjectSchmidt-Lanterman incisure
dc.subjectconnexin 29
dc.subjectconnexin 32
dc.subjectconnexin 43
dc.subjectconnexin 46
dc.subjectgap junction protein
dc.subjectgap junction protein beta 1
dc.subjectmaltose binding protein
dc.subjectmyelin
dc.subjectperipheral myelin protein 22
dc.subjectpotassium channel Kv1.1
dc.subjectunclassified drug
dc.subjectaction potential
dc.subjectcentral nervous system
dc.subjectchromosome Xq
dc.subjectdemyelinating neuropathy
dc.subjectdemyelination
dc.subjectelectrostimulation
dc.subjectfoot malformation
dc.subjectgap junction
dc.subjectgene mutation
dc.subjectgene targeting
dc.subjectgene therapy
dc.subjecthereditary motor sensory neuropathy
dc.subjecthuman
dc.subjectimmunocytochemistry
dc.subjectimmunofluorescence test
dc.subjectimmunogold labeling
dc.subjectmotor performance
dc.subjectmuscle atrophy
dc.subjectmuscular dystrophy
dc.subjectmyelin sheath
dc.subjectnerve conduction
dc.subjectnerve conduction velocity
dc.subjectnerve degeneration
dc.subjectnonhuman
dc.subjectperception deafness
dc.subjectperipheral neuropathy
dc.subjectprotein expression
dc.subjectSchwann cell
dc.subjectsensory nerve cell
dc.subjectShort Survey
dc.subjectX chromosome linked disorder
dc.titleRole of connexin-based gap junction channels in communication of myelin sheath in schwann cells
dc.typeShort Survey


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