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The Journal of Membrane Biology

, Volume 251, Issue 4, pp 535–550 | Cite as

Biochemical and Functional Interplay Between Ion Channels and the Components of the Dystrophin-Associated Glycoprotein Complex

Topical Review

Abstract

Dystrophin is a cytoskeleton-linked membrane protein that binds to a larger multiprotein assembly called the dystrophin-associated glycoprotein complex (DGC). The deficiency of dystrophin or the components of the DGC results in the loss of connection between the cytoskeleton and the extracellular matrix with significant pathophysiological implications in skeletal and cardiac muscle as well as in the nervous system. Although the DGC plays an important role in maintaining membrane stability, it can also be considered as a versatile and flexible molecular complex that contribute to the cellular organization and dynamics of a variety of proteins at specific locations in the plasma membrane. This review deals with the role of the DGC in transmembrane signaling by forming supramolecular assemblies for regulating ion channel localization and activity. These interactions are relevant for cell homeostasis, and its alterations may play a significant role in the etiology and pathogenesis of various disorders affecting muscle and nerve function.

Keywords

DGC Dystrophin Ion channels NaV channels CaV channels TRP 

Notes

Acknowledgements

This work was partially supported by funds from The National Council for Science and Technology (Conacyt, Mexico; Grant No. 221660) to R.F.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Supplementary material

232_2018_36_MOESM1_ESM.jpg (135 kb)
Suppl Fig 1. Molecular organization of voltage-gated Ca2+ (CaV), Na+ (NaV) and inward rectifying K+ (Kir) channels. The pore-forming α-subunit of NaV channels contains four domains each with six transmembrane segments with the N- and C-termini located in the cytoplasm. Ancillary β-subunits are single transmembrane proteins that co-assembles with the NaV α-subunit. CaV channels show a similar topology to NaV channels in their α-subunits but can be associated with different auxiliary subunits named α2δ, β and occasionally a γ-subunit with four transmembrane segments. BK channels comprise four ion-conducting α-subunits and in some tissues β auxiliary subunits. The α-subunits alone are sufficient to form a functional channel. These channels have an extra transmembrane domain that places its amino-terminal outside the cell. It also has a large intracellular carboxyl-terminus region that confers Ca2+ sensitivity to the channel complex. Kir channels contain two transmembrane (2 TM) and one pore-forming domain. The 2 TM domains assemble into a tetrameric ion-conducting Kir channel. Last, the membrane topology of cation channels of the transient receptor potential canonical (TRPC) family consists of six transmembrane spanning segments that are linked by short extracellular or intracellular loops (JPG 136 KB)
232_2018_36_MOESM2_ESM.jpg (54 kb)
Suppl Fig 2. Molecular organization of voltage-gated Ca2+ (CaV), Na+ (NaV) and inward rectifying K+ (Kir) channels. The pore-forming α-subunit of NaV channels contains four domains each with six transmembrane segments with the N- and C-termini located in the cytoplasm. Ancillary β-subunits are single transmembrane proteins that co-assembles with the NaV α-subunit. CaV channels show a similar topology to NaV channels in their α-subunits but can be associated with different auxiliary subunits named α2δ, β and occasionally a γ-subunit with four transmembrane segments. BK channels comprise four ion-conducting α-subunits and in some tissues β auxiliary subunits. The α-subunits alone are sufficient to form a functional channel. These channels have an extra transmembrane domain that places its amino-terminal outside the cell. It also has a large intracellular carboxyl-terminus region that confers Ca2+ sensitivity to the channel complex. Kir channels contain two transmembrane (2 TM) and one pore-forming domain. The 2 TM domains assemble into a tetrameric ion-conducting Kir channel. Last, the membrane topology of cation channels of the transient receptor potential canonical (TRPC) family consists of six transmembrane spanning segments that are linked by short extracellular or intracellular loops (JPG 54 KB)

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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Molecular Biology and Histocompatibility“Dr. Manuel Gea González” General HospitalMexico CityMexico
  2. 2.Faculty of Superior Studies IztacalaNational Autonomous University of Mexico (UNAM)TlalnepantlaMexico
  3. 3.Department of Cell BiologyCenter for Research and Advanced Studies of the National Polytechnic Institute (Cinvestav-IPN)Mexico CityMexico

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