Targeting ER stress/ER stress response in myopathies
There is more skeletal muscle tissue in the body than any other tissue and, as it is the organ of the majority of metabolic activity, muscle defect can affect the health of the entire body. Endoplasmic reticulum (ER) stress due to defects in protein folding/degradation balance, altered calcium and l...
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| Format: | Article |
| Language: | English |
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Elsevier
2019-09-01
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| Series: | Redox Biology |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213231719304239 |
| _version_ | 1818304991243272192 |
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| author | Ester Zito |
| author_facet | Ester Zito |
| author_sort | Ester Zito |
| collection | DOAJ |
| description | There is more skeletal muscle tissue in the body than any other tissue and, as it is the organ of the majority of metabolic activity, muscle defect can affect the health of the entire body. Endoplasmic reticulum (ER) stress due to defects in protein folding/degradation balance, altered calcium and lipid levels and alterations in ER-mitochondria contacts has recently been recognised as the pathogenic cause of many different myopathies. In addition, a maladaptive ER stress response triggered by ER stress and mediated by three ER stress sensors (PERK, IRE1 and ATF6) is involved in a failure to relieve muscle tissue from this stress. Targeting ER stress and the ER stress response pathway offers a broad range of opportunities for treating myopathies but, as the inhibition of the three ER stress sensors may not be safe because it could lead to unexpected effects; it therefore calls for careful analysis of the changes in downstream signal transduction in the different myopathies so these sub-pathways can be pharmacologically targeted. This review summarises the known inhibitors of the ER stress response and the successful results obtained using some of them in mouse models of muscle diseases caused by ER stress/ER stress response. |
| first_indexed | 2024-12-13T06:19:29Z |
| format | Article |
| id | doaj.art-66bfa9b5dc3f4edc8c009c7dfe468044 |
| institution | Directory Open Access Journal |
| issn | 2213-2317 |
| language | English |
| last_indexed | 2024-12-13T06:19:29Z |
| publishDate | 2019-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Redox Biology |
| spelling | doaj.art-66bfa9b5dc3f4edc8c009c7dfe4680442022-12-21T23:56:53ZengElsevierRedox Biology2213-23172019-09-0126Targeting ER stress/ER stress response in myopathiesEster Zito0Dulbecco Telethon Institute at IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Milan, ItalyThere is more skeletal muscle tissue in the body than any other tissue and, as it is the organ of the majority of metabolic activity, muscle defect can affect the health of the entire body. Endoplasmic reticulum (ER) stress due to defects in protein folding/degradation balance, altered calcium and lipid levels and alterations in ER-mitochondria contacts has recently been recognised as the pathogenic cause of many different myopathies. In addition, a maladaptive ER stress response triggered by ER stress and mediated by three ER stress sensors (PERK, IRE1 and ATF6) is involved in a failure to relieve muscle tissue from this stress. Targeting ER stress and the ER stress response pathway offers a broad range of opportunities for treating myopathies but, as the inhibition of the three ER stress sensors may not be safe because it could lead to unexpected effects; it therefore calls for careful analysis of the changes in downstream signal transduction in the different myopathies so these sub-pathways can be pharmacologically targeted. This review summarises the known inhibitors of the ER stress response and the successful results obtained using some of them in mouse models of muscle diseases caused by ER stress/ER stress response.http://www.sciencedirect.com/science/article/pii/S2213231719304239 |
| spellingShingle | Ester Zito Targeting ER stress/ER stress response in myopathies Redox Biology |
| title | Targeting ER stress/ER stress response in myopathies |
| title_full | Targeting ER stress/ER stress response in myopathies |
| title_fullStr | Targeting ER stress/ER stress response in myopathies |
| title_full_unstemmed | Targeting ER stress/ER stress response in myopathies |
| title_short | Targeting ER stress/ER stress response in myopathies |
| title_sort | targeting er stress er stress response in myopathies |
| url | http://www.sciencedirect.com/science/article/pii/S2213231719304239 |
| work_keys_str_mv | AT esterzito targetingerstresserstressresponseinmyopathies |