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The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle

This review details the role of dystrophin and the dystrophin associated proteins (DAPs) in the vascular smooth muscle. Dystrophin is most comprehensively studied in the skeletal muscle due to serious symptoms found related to the skeletal muscle of patients with muscular dystrophy. Mutations in the...

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Autores principales: Kaplan, Katherine M., Morgan, Kathleen G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9732661/
https://www.ncbi.nlm.nih.gov/pubmed/36505053
http://dx.doi.org/10.3389/fphys.2022.1059021
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author Kaplan, Katherine M.
Morgan, Kathleen G.
author_facet Kaplan, Katherine M.
Morgan, Kathleen G.
author_sort Kaplan, Katherine M.
collection PubMed
description This review details the role of dystrophin and the dystrophin associated proteins (DAPs) in the vascular smooth muscle. Dystrophin is most comprehensively studied in the skeletal muscle due to serious symptoms found related to the skeletal muscle of patients with muscular dystrophy. Mutations in the dystrophin gene, or DAPs genes, result in a wide range of muscular dystrophies. In skeletal muscle, dystrophin is known to act to as a cytoskeletal stabilization protein and protects cells against contraction-induced damage. In skeletal muscle, dystrophin stabilizes the plasma membrane by transmitting forces generated by sarcomeric contraction to the extracellular matrix (ECM). Dystrophin is a scaffold that binds the dystroglycan complex (DGC) and has many associated proteins (DAPs). These DAPs include sarcoglycans, syntrophins, dystroglycans, dystrobrevin, neuronal nitric oxide synthase, and caveolins. The DAPs provide biomechanical support to the skeletal or cardiac plasma membrane during contraction, and loss of one or several of these DAPs leads to plasma membrane fragility. Dystrophin is expressed near the plasma membrane of all muscles, including cardiac and vascular smooth muscle, and some neurons. Dystrophic mice have noted biomechanical irregularities in the carotid arteries and spontaneous motor activity in portal vein altered when compared to wild type mice. Additionally, some studies suggest the vasculature of patients and animal models with muscular dystrophy is abnormal. Although the function of dystrophin and the DAPs in vascular smooth muscle is not thoroughly established in the field, this review makes the point that these proteins are expressed, and important and further study is warranted.
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spelling pubmed-97326612022-12-10 The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle Kaplan, Katherine M. Morgan, Kathleen G. Front Physiol Physiology This review details the role of dystrophin and the dystrophin associated proteins (DAPs) in the vascular smooth muscle. Dystrophin is most comprehensively studied in the skeletal muscle due to serious symptoms found related to the skeletal muscle of patients with muscular dystrophy. Mutations in the dystrophin gene, or DAPs genes, result in a wide range of muscular dystrophies. In skeletal muscle, dystrophin is known to act to as a cytoskeletal stabilization protein and protects cells against contraction-induced damage. In skeletal muscle, dystrophin stabilizes the plasma membrane by transmitting forces generated by sarcomeric contraction to the extracellular matrix (ECM). Dystrophin is a scaffold that binds the dystroglycan complex (DGC) and has many associated proteins (DAPs). These DAPs include sarcoglycans, syntrophins, dystroglycans, dystrobrevin, neuronal nitric oxide synthase, and caveolins. The DAPs provide biomechanical support to the skeletal or cardiac plasma membrane during contraction, and loss of one or several of these DAPs leads to plasma membrane fragility. Dystrophin is expressed near the plasma membrane of all muscles, including cardiac and vascular smooth muscle, and some neurons. Dystrophic mice have noted biomechanical irregularities in the carotid arteries and spontaneous motor activity in portal vein altered when compared to wild type mice. Additionally, some studies suggest the vasculature of patients and animal models with muscular dystrophy is abnormal. Although the function of dystrophin and the DAPs in vascular smooth muscle is not thoroughly established in the field, this review makes the point that these proteins are expressed, and important and further study is warranted. Frontiers Media S.A. 2022-11-25 /pmc/articles/PMC9732661/ /pubmed/36505053 http://dx.doi.org/10.3389/fphys.2022.1059021 Text en Copyright © 2022 Kaplan and Morgan. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Physiology
Kaplan, Katherine M.
Morgan, Kathleen G.
The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
title The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
title_full The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
title_fullStr The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
title_full_unstemmed The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
title_short The importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
title_sort importance of dystrophin and the dystrophin associated proteins in vascular smooth muscle
topic Physiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9732661/
https://www.ncbi.nlm.nih.gov/pubmed/36505053
http://dx.doi.org/10.3389/fphys.2022.1059021
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