mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases
Mitochondrial dysfunction is a key pathological event in many diseases. Its role in energy production, calcium homeostasis, apoptosis regulation, and reactive oxygen species (ROS) balance render mitochondria essential for cell survival and fitness. However, there are no effective treatments for most...
| Autores: | , , , , , , , , , , , , |
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| Formato: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2023 |
| País: | España |
| Recursos: | Consejo Superior de Investigaciones Científicas (CSIC) |
| Repositorio: | DIGITAL.CSIC. Repositorio Institucional del CSIC |
| OAI Identifier: | oai:digital.csic.es:10261/336881 |
| Acesso em linha: | http://hdl.handle.net/10261/336881 |
| Access Level: | acceso abierto |
| Palavra-chave: | Mtochondria Proteostasis Mitochondrial unfolded protein response Mitochondrial biogenesis Therapeutic target Neurodegenerative diseases Mitochondrial diseases Ageing Metabolic diseases Cardiovascular diseases Cancer |
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mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial DiseasesCilleros-Holgado, PaulaGómez-Fernández, DavidPiñero-Perez, RocíoReche-López, DianaÁlvarez-Córdoba, MónicaMunuera, ManuelTalaverón-Rey, MartaPovea-Cabello, SulevaSuárez-Carrillo, AlejandraRomero-González, AnaSuarez-Rivero, Juan M.Romero-Domínguez, José ManuelSánchez-Alcázar, José AntonioMtochondriaProteostasisMitochondrial unfolded protein responseMitochondrial biogenesisTherapeutic targetNeurodegenerative diseasesMitochondrial diseasesAgeingMetabolic diseasesCardiovascular diseasesCancerMitochondrial dysfunction is a key pathological event in many diseases. Its role in energy production, calcium homeostasis, apoptosis regulation, and reactive oxygen species (ROS) balance render mitochondria essential for cell survival and fitness. However, there are no effective treatments for most primary and secondary mitochondrial diseases to this day. Therefore, new therapeutic approaches, such as the modulation of the mitochondrial unfolded protein response (mtUPR), are being explored. mtUPRs englobe several compensatory processes related to proteostasis and antioxidant system mechanisms. mtUPR activation, through an overcompensation for mild intracellular stress, promotes cell homeostasis and improves lifespan and disease alterations in biological models of mitochondrial dysfunction in age-related diseases, cardiopathies, metabolic disorders, and primary mitochondrial diseases. Although mtUPR activation is a promising therapeutic option for many pathological conditions, its activation could promote tumor progression in cancer patients, and its overactivation could lead to non-desired side effects, such as the increased heteroplasmy of mitochondrial DNA mutations. In this review, we present the most recent data about mtUPR modulation as a therapeutic approach, its role in diseases, and its potential negative consequences in specific pathological situations.This project was supported by the FIS PI16/00786 (2016) and FIS PI19/00377 (2019) grants; the Ministerio de Sanidad, Spain; and the Fondo Europeo de Desarrollo Regional (FEDER Unión Europea), Spanish Ministry of Education, Culture, and Sport. This activity was co-financed by the European Regional Development Fund (ERDF) and by the Regional Ministry of Economic Transformation, Industry, Knowledge, and Universities of the Junta de Andalucía, within the framework of the ERDF Andalusia operational program 2014–2020 Thematic objective “01—Reinforcement of research, technological development and innovation” through the reference research project CTS-5725 and PY18-850.Multidisciplinary Digital Publishing InstituteMinisterio de Sanidad (España)European CommissionMinisterio de Educación, Cultura y Deporte (España)Junta de AndalucíaConsejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]2023202320232023info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_dcae04bcPublisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/336881reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttp://dx.doi.org/10.3390/ijms24021482Síinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/3368812026-05-22T06:33:51Z |
| dc.title.none.fl_str_mv |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| title |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| spellingShingle |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases Cilleros-Holgado, Paula Mtochondria Proteostasis Mitochondrial unfolded protein response Mitochondrial biogenesis Therapeutic target Neurodegenerative diseases Mitochondrial diseases Ageing Metabolic diseases Cardiovascular diseases Cancer |
| title_short |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| title_full |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| title_fullStr |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| title_full_unstemmed |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| title_sort |
mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases |
| dc.creator.none.fl_str_mv |
Cilleros-Holgado, Paula Gómez-Fernández, David Piñero-Perez, Rocío Reche-López, Diana Álvarez-Córdoba, Mónica Munuera, Manuel Talaverón-Rey, Marta Povea-Cabello, Suleva Suárez-Carrillo, Alejandra Romero-González, Ana Suarez-Rivero, Juan M. Romero-Domínguez, José Manuel Sánchez-Alcázar, José Antonio |
| author |
Cilleros-Holgado, Paula |
| author_facet |
Cilleros-Holgado, Paula Gómez-Fernández, David Piñero-Perez, Rocío Reche-López, Diana Álvarez-Córdoba, Mónica Munuera, Manuel Talaverón-Rey, Marta Povea-Cabello, Suleva Suárez-Carrillo, Alejandra Romero-González, Ana Suarez-Rivero, Juan M. Romero-Domínguez, José Manuel Sánchez-Alcázar, José Antonio |
| author_role |
author |
| author2 |
Gómez-Fernández, David Piñero-Perez, Rocío Reche-López, Diana Álvarez-Córdoba, Mónica Munuera, Manuel Talaverón-Rey, Marta Povea-Cabello, Suleva Suárez-Carrillo, Alejandra Romero-González, Ana Suarez-Rivero, Juan M. Romero-Domínguez, José Manuel Sánchez-Alcázar, José Antonio |
| author2_role |
author author author author author author author author author author author author |
| dc.contributor.none.fl_str_mv |
Ministerio de Sanidad (España) European Commission Ministerio de Educación, Cultura y Deporte (España) Junta de Andalucía Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72] |
| dc.subject.none.fl_str_mv |
Mtochondria Proteostasis Mitochondrial unfolded protein response Mitochondrial biogenesis Therapeutic target Neurodegenerative diseases Mitochondrial diseases Ageing Metabolic diseases Cardiovascular diseases Cancer |
| topic |
Mtochondria Proteostasis Mitochondrial unfolded protein response Mitochondrial biogenesis Therapeutic target Neurodegenerative diseases Mitochondrial diseases Ageing Metabolic diseases Cardiovascular diseases Cancer |
| description |
Mitochondrial dysfunction is a key pathological event in many diseases. Its role in energy production, calcium homeostasis, apoptosis regulation, and reactive oxygen species (ROS) balance render mitochondria essential for cell survival and fitness. However, there are no effective treatments for most primary and secondary mitochondrial diseases to this day. Therefore, new therapeutic approaches, such as the modulation of the mitochondrial unfolded protein response (mtUPR), are being explored. mtUPRs englobe several compensatory processes related to proteostasis and antioxidant system mechanisms. mtUPR activation, through an overcompensation for mild intracellular stress, promotes cell homeostasis and improves lifespan and disease alterations in biological models of mitochondrial dysfunction in age-related diseases, cardiopathies, metabolic disorders, and primary mitochondrial diseases. Although mtUPR activation is a promising therapeutic option for many pathological conditions, its activation could promote tumor progression in cancer patients, and its overactivation could lead to non-desired side effects, such as the increased heteroplasmy of mitochondrial DNA mutations. In this review, we present the most recent data about mtUPR modulation as a therapeutic approach, its role in diseases, and its potential negative consequences in specific pathological situations. |
| publishDate |
2023 |
| dc.date.none.fl_str_mv |
2023 2023 2023 2023 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article http://purl.org/coar/resource_type/c_dcae04bc Publisher's version info:eu-repo/semantics/publishedVersion |
| format |
article |
| status_str |
publishedVersion |
| dc.identifier.none.fl_str_mv |
http://hdl.handle.net/10261/336881 |
| url |
http://hdl.handle.net/10261/336881 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
| dc.relation.none.fl_str_mv |
http://dx.doi.org/10.3390/ijms24021482 Sí |
| dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess |
| eu_rights_str_mv |
openAccess |
| dc.publisher.none.fl_str_mv |
Multidisciplinary Digital Publishing Institute |
| publisher.none.fl_str_mv |
Multidisciplinary Digital Publishing Institute |
| dc.source.none.fl_str_mv |
reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC instname:Consejo Superior de Investigaciones Científicas (CSIC) |
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Consejo Superior de Investigaciones Científicas (CSIC) |
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DIGITAL.CSIC. Repositorio Institucional del CSIC |
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DIGITAL.CSIC. Repositorio Institucional del CSIC |
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1869410825270198272 |
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15.812429 |