Structural and signaling proteins in the Z-disk and their role in cardiomyopathies.

Z-disk protein alpha-actinin cardiomyopathy desmin (DES) filamin C (FLNC) myopalladin (MYPN) pathogenic variant titin (TTN)

Journal

Frontiers in physiology
ISSN: 1664-042X
Titre abrégé: Front Physiol
Pays: Switzerland
ID NLM: 101549006

Informations de publication

Date de publication:
2023
Historique:
received: 13 01 2023
accepted: 21 02 2023
entrez: 20 3 2023
pubmed: 21 3 2023
medline: 21 3 2023
Statut: epublish

Résumé

The sarcomere is the smallest functional unit of muscle contraction. It is delineated by a protein-rich structure known as the Z-disk, alternating with M-bands. The Z-disk anchors the actin-rich thin filaments and plays a crucial role in maintaining the mechanical stability of the cardiac muscle. A multitude of proteins interact with each other at the Z-disk and they regulate the mechanical properties of the thin filaments. Over the past 2 decades, the role of the Z-disk in cardiac muscle contraction has been assessed widely, however, the impact of genetic variants in Z-disk proteins has still not been fully elucidated. This review discusses the various Z-disk proteins (alpha-actinin, filamin C, titin, muscle LIM protein, telethonin, myopalladin, nebulette, and nexilin) and Z-disk-associated proteins (desmin, and obscurin) and their role in cardiac structural stability and intracellular signaling. This review further explores how genetic variants of Z-disk proteins are linked to inherited cardiac conditions termed cardiomyopathies.

Identifiants

pubmed: 36935760
doi: 10.3389/fphys.2023.1143858
pii: 1143858
pmc: PMC10017460
doi:

Types de publication

Journal Article Review

Langues

eng

Pagination

1143858

Informations de copyright

Copyright © 2023 Noureddine and Gehmlich.

Déclaration de conflit d'intérêts

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Maya Noureddine (M)

Institute of Cardiovascular Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom.

Katja Gehmlich (K)

Institute of Cardiovascular Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom.
Cardiovascular Medicine, Radcliffe Department of Medicine and British Heart Foundation Centre of Research Excellence Oxford, University of Oxford, Oxford, United Kingdom.

Classifications MeSH