Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders

Dysfunction of cellular homeostasis can lead to misfolding of proteins thus acquiring conformations prone to polymerization into pathological aggregates. This process is associated with several disorders, including neurodegenerative diseases, such as Parkinson’s disease (PD), and endoplasmic reticul...

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Autores principales:	Francisco J. Padilla-Godínez, Rodrigo Ramos-Acevedo, Hilda Angélica Martínez-Becerril, Luis D. Bernal-Conde, Jerónimo F. Garrido-Figueroa, Marcia Hiriart, Adriana Hernández-López, Rubén Argüero-Sánchez, Francesco Callea, Magdalena Guerra-Crespo
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	protein misfolding protein aggregation alpha-synuclein Parkinson’s disease endoplasmic reticulum storage disease alpha-1-antitrypsin Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/d5f496136f354511a7825b325ace6e3f
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spelling	oai:doaj.org-article:d5f496136f354511a7825b325ace6e3f2021-11-25T17:56:55ZProtein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders10.3390/ijms2222124671422-00671661-6596https://doaj.org/article/d5f496136f354511a7825b325ace6e3f2021-11-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/22/12467https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Dysfunction of cellular homeostasis can lead to misfolding of proteins thus acquiring conformations prone to polymerization into pathological aggregates. This process is associated with several disorders, including neurodegenerative diseases, such as Parkinson’s disease (PD), and endoplasmic reticulum storage disorders (ERSDs), like alpha-1-antitrypsin deficiency (AATD) and hereditary hypofibrinogenemia with hepatic storage (HHHS). Given the shared pathophysiological mechanisms involved in such conditions, it is necessary to deepen our understanding of the basic principles of misfolding and aggregation akin to these diseases which, although heterogeneous in symptomatology, present similarities that could lead to potential mutual treatments. Here, we review: (i) the pathological bases leading to misfolding and aggregation of proteins involved in PD, AATD, and HHHS: alpha-synuclein, alpha-1-antitrypsin, and fibrinogen, respectively, (ii) the evidence linking each protein aggregation to the stress mechanisms occurring in the endoplasmic reticulum (ER) of each pathology, (iii) a comparison of the mechanisms related to dysfunction of proteostasis and regulation of homeostasis between the diseases (such as the unfolded protein response and/or autophagy), (iv) and clinical perspectives regarding possible common treatments focused on improving the defensive responses to protein aggregation for diseases as different as PD, and ERSDs.Francisco J. Padilla-GodínezRodrigo Ramos-AcevedoHilda Angélica Martínez-BecerrilLuis D. Bernal-CondeJerónimo F. Garrido-FigueroaMarcia HiriartAdriana Hernández-LópezRubén Argüero-SánchezFrancesco CalleaMagdalena Guerra-CrespoMDPI AGarticleprotein misfoldingprotein aggregationalpha-synucleinParkinson’s diseaseendoplasmic reticulum storage diseasealpha-1-antitrypsinBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 12467, p 12467 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	protein misfolding protein aggregation alpha-synuclein Parkinson’s disease endoplasmic reticulum storage disease alpha-1-antitrypsin Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	protein misfolding protein aggregation alpha-synuclein Parkinson’s disease endoplasmic reticulum storage disease alpha-1-antitrypsin Biology (General) QH301-705.5 Chemistry QD1-999 Francisco J. Padilla-Godínez Rodrigo Ramos-Acevedo Hilda Angélica Martínez-Becerril Luis D. Bernal-Conde Jerónimo F. Garrido-Figueroa Marcia Hiriart Adriana Hernández-López Rubén Argüero-Sánchez Francesco Callea Magdalena Guerra-Crespo Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders
description	Dysfunction of cellular homeostasis can lead to misfolding of proteins thus acquiring conformations prone to polymerization into pathological aggregates. This process is associated with several disorders, including neurodegenerative diseases, such as Parkinson’s disease (PD), and endoplasmic reticulum storage disorders (ERSDs), like alpha-1-antitrypsin deficiency (AATD) and hereditary hypofibrinogenemia with hepatic storage (HHHS). Given the shared pathophysiological mechanisms involved in such conditions, it is necessary to deepen our understanding of the basic principles of misfolding and aggregation akin to these diseases which, although heterogeneous in symptomatology, present similarities that could lead to potential mutual treatments. Here, we review: (i) the pathological bases leading to misfolding and aggregation of proteins involved in PD, AATD, and HHHS: alpha-synuclein, alpha-1-antitrypsin, and fibrinogen, respectively, (ii) the evidence linking each protein aggregation to the stress mechanisms occurring in the endoplasmic reticulum (ER) of each pathology, (iii) a comparison of the mechanisms related to dysfunction of proteostasis and regulation of homeostasis between the diseases (such as the unfolded protein response and/or autophagy), (iv) and clinical perspectives regarding possible common treatments focused on improving the defensive responses to protein aggregation for diseases as different as PD, and ERSDs.
format	article
author	Francisco J. Padilla-Godínez Rodrigo Ramos-Acevedo Hilda Angélica Martínez-Becerril Luis D. Bernal-Conde Jerónimo F. Garrido-Figueroa Marcia Hiriart Adriana Hernández-López Rubén Argüero-Sánchez Francesco Callea Magdalena Guerra-Crespo
author_facet	Francisco J. Padilla-Godínez Rodrigo Ramos-Acevedo Hilda Angélica Martínez-Becerril Luis D. Bernal-Conde Jerónimo F. Garrido-Figueroa Marcia Hiriart Adriana Hernández-López Rubén Argüero-Sánchez Francesco Callea Magdalena Guerra-Crespo
author_sort	Francisco J. Padilla-Godínez
title	Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders
title_short	Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders
title_full	Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders
title_fullStr	Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders
title_full_unstemmed	Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders
title_sort	protein misfolding and aggregation: the relatedness between parkinson’s disease and hepatic endoplasmic reticulum storage disorders
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/d5f496136f354511a7825b325ace6e3f
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Protein Misfolding and Aggregation: The Relatedness between Parkinson’s Disease and Hepatic Endoplasmic Reticulum Storage Disorders

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