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Is Desmin Propensity to Aggregate Part of its Protective Function?

Desmin is the major protein component of the intermediate filaments (IFs) cytoskeleton in muscle cells, including cardiac. The accumulation of cleaved and misfolded desmin is a cellular hallmark of heart failure (HF). These desmin alterations are reversed by therapy, suggesting a causal role for the...

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Autores principales: Singh, Sonia R., Kadioglu, Hikmet, Patel, Krishna, Carrier, Lucie, Agnetti, Giulio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072738/
https://www.ncbi.nlm.nih.gov/pubmed/32093415
http://dx.doi.org/10.3390/cells9020491
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author Singh, Sonia R.
Kadioglu, Hikmet
Patel, Krishna
Carrier, Lucie
Agnetti, Giulio
author_facet Singh, Sonia R.
Kadioglu, Hikmet
Patel, Krishna
Carrier, Lucie
Agnetti, Giulio
author_sort Singh, Sonia R.
collection PubMed
description Desmin is the major protein component of the intermediate filaments (IFs) cytoskeleton in muscle cells, including cardiac. The accumulation of cleaved and misfolded desmin is a cellular hallmark of heart failure (HF). These desmin alterations are reversed by therapy, suggesting a causal role for the IFs in the development of HF. Though IFs are known to play a role in the protection from stress, a mechanistic model of how that occurs is currently lacking. On the other hand, the heart is uniquely suited to study the function of the IFs, due to its inherent, cyclic contraction. That is, HF can be used as a model to address how IFs afford protection from mechanical, and possibly redox, stress. In this review we provide a brief summary of the current views on the function of the IFs, focusing on desmin. We also propose a new model according to which the propensity of desmin to aggregate may have been selected during evolution as a way to dissipate excessive mechanical and possibly redox stress. According to this model, though desmin misfolding may afford protection from acute injury, the sustained or excessive accumulation of desmin aggregates could impair proteostasis and contribute to disease.
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spelling pubmed-70727382020-03-19 Is Desmin Propensity to Aggregate Part of its Protective Function? Singh, Sonia R. Kadioglu, Hikmet Patel, Krishna Carrier, Lucie Agnetti, Giulio Cells Review Desmin is the major protein component of the intermediate filaments (IFs) cytoskeleton in muscle cells, including cardiac. The accumulation of cleaved and misfolded desmin is a cellular hallmark of heart failure (HF). These desmin alterations are reversed by therapy, suggesting a causal role for the IFs in the development of HF. Though IFs are known to play a role in the protection from stress, a mechanistic model of how that occurs is currently lacking. On the other hand, the heart is uniquely suited to study the function of the IFs, due to its inherent, cyclic contraction. That is, HF can be used as a model to address how IFs afford protection from mechanical, and possibly redox, stress. In this review we provide a brief summary of the current views on the function of the IFs, focusing on desmin. We also propose a new model according to which the propensity of desmin to aggregate may have been selected during evolution as a way to dissipate excessive mechanical and possibly redox stress. According to this model, though desmin misfolding may afford protection from acute injury, the sustained or excessive accumulation of desmin aggregates could impair proteostasis and contribute to disease. MDPI 2020-02-20 /pmc/articles/PMC7072738/ /pubmed/32093415 http://dx.doi.org/10.3390/cells9020491 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Singh, Sonia R.
Kadioglu, Hikmet
Patel, Krishna
Carrier, Lucie
Agnetti, Giulio
Is Desmin Propensity to Aggregate Part of its Protective Function?
title Is Desmin Propensity to Aggregate Part of its Protective Function?
title_full Is Desmin Propensity to Aggregate Part of its Protective Function?
title_fullStr Is Desmin Propensity to Aggregate Part of its Protective Function?
title_full_unstemmed Is Desmin Propensity to Aggregate Part of its Protective Function?
title_short Is Desmin Propensity to Aggregate Part of its Protective Function?
title_sort is desmin propensity to aggregate part of its protective function?
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072738/
https://www.ncbi.nlm.nih.gov/pubmed/32093415
http://dx.doi.org/10.3390/cells9020491
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