Review
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Role of the Alpha-B-Crystallin Protein in Cardiomyopathic Disease
Version 1
: Received: 17 January 2024 / Approved: 18 January 2024 / Online: 18 January 2024 (10:30:11 CET)
A peer-reviewed article of this Preprint also exists.
Thorkelsson, A.; Chin, M.T. Role of the Alpha-B-Crystallin Protein in Cardiomyopathic Disease. Int. J. Mol. Sci. 2024, 25, 2826. Thorkelsson, A.; Chin, M.T. Role of the Alpha-B-Crystallin Protein in Cardiomyopathic Disease. Int. J. Mol. Sci. 2024, 25, 2826.
Abstract
Alpha-B-Crystallin, a member of the small heat shock family of proteins, has been implicated in a variety of cardiomyopathies and in normal cardiac homeostasis. It is known to function as a molecular chaperone, particularly for desmin, but also interacts with a wide variety of additional proteins. The molecular chaperone function is also enhanced by signal-dependent phosphorylation at specific residues under stress conditions. Naturally occurring mutations in CRYAB, the gene that encodes alpha-B-crystallin, have been suggested to alter ionic intermolecular interactions that affect dimerization and affect chaperone function. These mutations have been associated with myofibrillar myopathy, restrictive cardiomyopathy, and hypertrophic cardiomyopathy, and promote pathological hypertrophy through different mechanisms such as desmin aggregation, increased reductive stress or activation of calcineurin-NFAT signaling. This review will discuss the known mechanisms by which alpha-B-crystallin functions in cardiac homeostasis and the pathogenesis of cardiomyopathies and provide insight into potential future areas of exploration.
Keywords
alpha-B-crystallin; cryab; molecular chaperone; desminopathy; hypertrophic cardiomyopathy; dilated cardiomyopathy; restrictive cardiomyopathy; calcineurin; NFAT
Subject
Biology and Life Sciences, Biochemistry and Molecular Biology
Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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