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doi:10.1101/sqb.1996.061.01.067

Cited by 7 publications

(1 citation statement)

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“…The Cxs play a critical role in the functioning of the myelinating SCs. Indeed, mutations in the human gene gap junction protein beta 1 ( GJB1 ), which encodes Cx32, lead to the pathological phenotype of the X-chromosomal form of Charcot-Marie-Tooth (CMT1X or CMTX1), where inflammatory processes in peripheral nerves decrease conduction velocity of action potentials, leading to muscle atrophy (Bergoffen et al, 1993a; Fischbeck et al, 1996; Abrams et al, 2002). This disease is the second most common form of hereditary motor and sensory neuropathy, and there is no cure (Kleopa et al, 2012).…”

Section: Gap Junction Channels In Myelinating Schwann Cellsmentioning

confidence: 99%

Role of Connexin-Based Gap Junction Channels in Communication of Myelin Sheath in Schwann Cells

Cisterna

Arroyo

Puebla

2019

Front. Cell. Neurosci.

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Peripheral 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 K v 1, 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.

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Section: Gap Junction Channels In Myelinating Schwann Cellsmentioning

confidence: 99%

Role of Connexin-Based Gap Junction Channels in Communication of Myelin Sheath in Schwann Cells

Cisterna

Arroyo

Puebla

2019

Front. Cell. Neurosci.

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Connexins in Tumour Suppression and Cancer Therapy

Yamasaki

Omori

Krutovskikh

et al. 2007

Novartis Foundation Symposium 219 ‐ Gap Junction‐Mediated Intercellular Signalling in Health and Disease

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Sensitivity of the brain transcriptome to connexin ablation

Iacobaş

Urban-Maldonado

et al. 2005

Biochimica et Biophysica Acta (BBA) - Biomembranes

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Extensive studies on mice with total or partial disruption of either connexin43 (Cx43) or connexin32 (Cx32) have detected only subtle changes in central nervous system structure, growth, development, or function. We have used high density cDNA arrays to analyze the regulation, control, and coordination of the abundances of 7446 distinct transcripts in four brains, each of Cx43 null (K43), Cx43 heterozygous (H43), and Cx32 null (K32) mice as compared to the brains of wildtype (W) mice. The use of multiple samples allowed the determination of the statistical significance of gene regulation. Significantly regulated genes encoded proteins of all functional categories, extending beyond those that might be expected to depend on junctional communication. Moreover, we found a high degree of similarity between genes regulated in the K43 and H43 brains and a remarkable overlap between gene regulation in brains of K43 and K32. The regulated genes in both K43 and H43 brains showed an outstanding inverse coordination with the levels of expression of Cx43 in W brain, indicating that the regulated genes are largely predictable from their co-variance with Cx43 in the wildtype samples. These findings lead to the hypothesis that connexin expression may represent a central node in the regulation of gene expression patterns in brain.

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Cited by 7 publications

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Role of Connexin-Based Gap Junction Channels in Communication of Myelin Sheath in Schwann Cells

Role of Connexin-Based Gap Junction Channels in Communication of Myelin Sheath in Schwann Cells

Connexins in Tumour Suppression and Cancer Therapy

Sensitivity of the brain transcriptome to connexin ablation

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