PMID- 30881289
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 1662-5102 (Print)
IS  - 1662-5102 (Electronic)
IS  - 1662-5102 (Linking)
VI  - 13
DP  - 2019
TI  - Role of Connexin-Based Gap Junction Channels in Communication of Myelin Sheath in 
      Schwann Cells.
PG  - 69
LID - 10.3389/fncel.2019.00069 [doi]
LID - 69
AB  - 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.
FAU - Cisterna, Bruno A
AU  - Cisterna BA
AD  - Escuela de Medicina, Universidad de Talca, Talca, Chile.
AD  - Centro para el Desarrollo de la Nanociencia y Nanotecnologia (CEDENNA), 
      Universidad de Santiago de Chile, Santiago, Chile.
FAU - Arroyo, Pablo
AU  - Arroyo P
AD  - Facultad de Medicina, Pontificia Universidad Catolica de Chile, Santiago, Chile.
FAU - Puebla, Carlos
AU  - Puebla C
AD  - Instituto de Ciencias Biomedicas, Facultad de Ciencias de la Salud, Universidad 
      Autonoma de Chile, Santiago, Chile.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20190301
PL  - Switzerland
TA  - Front Cell Neurosci
JT  - Frontiers in cellular neuroscience
JID - 101477935
PMC - PMC6405416
OTO - NOTNLM
OT  - CMTX1
OT  - Charcot-Marie-Tooth disease
OT  - Schmidt-Lanterman incisure
OT  - connexins
OT  - gap junction channels
OT  - myelin sheath
EDAT- 2019/03/19 06:00
MHDA- 2019/03/19 06:01
PMCR- 2019/01/01
CRDT- 2019/03/19 06:00
PHST- 2018/09/15 00:00 [received]
PHST- 2019/02/12 00:00 [accepted]
PHST- 2019/03/19 06:00 [entrez]
PHST- 2019/03/19 06:00 [pubmed]
PHST- 2019/03/19 06:01 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fncel.2019.00069 [doi]
PST - epublish
SO  - Front Cell Neurosci. 2019 Mar 1;13:69. doi: 10.3389/fncel.2019.00069. eCollection 
      2019.