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Neuroserpin: structure, function, physiology and pathology
Neuroserpin is a serine protease inhibitor identified in a search for proteins implicated in neuronal axon growth and synapse formation. Since its discovery over 30 years ago, it has been the focus of active research. Many efforts have concentrated in elucidating its neuroprotective role in brain is...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer International Publishing
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8558161/ https://www.ncbi.nlm.nih.gov/pubmed/34405255 http://dx.doi.org/10.1007/s00018-021-03907-6 |
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author | D’Acunto, Emanuela Fra, Annamaria Visentin, Cristina Manno, Mauro Ricagno, Stefano Galliciotti, Giovanna Miranda, Elena |
author_facet | D’Acunto, Emanuela Fra, Annamaria Visentin, Cristina Manno, Mauro Ricagno, Stefano Galliciotti, Giovanna Miranda, Elena |
author_sort | D’Acunto, Emanuela |
collection | PubMed |
description | Neuroserpin is a serine protease inhibitor identified in a search for proteins implicated in neuronal axon growth and synapse formation. Since its discovery over 30 years ago, it has been the focus of active research. Many efforts have concentrated in elucidating its neuroprotective role in brain ischemic lesions, the structural bases of neuroserpin conformational change and the effects of neuroserpin polymers that underlie the neurodegenerative disease FENIB (familial encephalopathy with neuroserpin inclusion bodies), but the investigation of the physiological roles of neuroserpin has increased over the last years. In this review, we present an updated and critical revision of the current literature dealing with neuroserpin, covering all aspects of research including the expression and physiological roles of neuroserpin, both inside and outside the nervous system; its inhibitory and non-inhibitory mechanisms of action; the molecular structure of the monomeric and polymeric conformations of neuroserpin, including a detailed description of the polymerisation mechanism; and the involvement of neuroserpin in human disease, with particular emphasis on FENIB. Finally, we briefly discuss the identification by genome-wide screening of novel neuroserpin variants and their possible pathogenicity. |
format | Online Article Text |
id | pubmed-8558161 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Springer International Publishing |
record_format | MEDLINE/PubMed |
spelling | pubmed-85581612021-11-15 Neuroserpin: structure, function, physiology and pathology D’Acunto, Emanuela Fra, Annamaria Visentin, Cristina Manno, Mauro Ricagno, Stefano Galliciotti, Giovanna Miranda, Elena Cell Mol Life Sci Review Neuroserpin is a serine protease inhibitor identified in a search for proteins implicated in neuronal axon growth and synapse formation. Since its discovery over 30 years ago, it has been the focus of active research. Many efforts have concentrated in elucidating its neuroprotective role in brain ischemic lesions, the structural bases of neuroserpin conformational change and the effects of neuroserpin polymers that underlie the neurodegenerative disease FENIB (familial encephalopathy with neuroserpin inclusion bodies), but the investigation of the physiological roles of neuroserpin has increased over the last years. In this review, we present an updated and critical revision of the current literature dealing with neuroserpin, covering all aspects of research including the expression and physiological roles of neuroserpin, both inside and outside the nervous system; its inhibitory and non-inhibitory mechanisms of action; the molecular structure of the monomeric and polymeric conformations of neuroserpin, including a detailed description of the polymerisation mechanism; and the involvement of neuroserpin in human disease, with particular emphasis on FENIB. Finally, we briefly discuss the identification by genome-wide screening of novel neuroserpin variants and their possible pathogenicity. Springer International Publishing 2021-08-17 2021 /pmc/articles/PMC8558161/ /pubmed/34405255 http://dx.doi.org/10.1007/s00018-021-03907-6 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Review D’Acunto, Emanuela Fra, Annamaria Visentin, Cristina Manno, Mauro Ricagno, Stefano Galliciotti, Giovanna Miranda, Elena Neuroserpin: structure, function, physiology and pathology |
title | Neuroserpin: structure, function, physiology and pathology |
title_full | Neuroserpin: structure, function, physiology and pathology |
title_fullStr | Neuroserpin: structure, function, physiology and pathology |
title_full_unstemmed | Neuroserpin: structure, function, physiology and pathology |
title_short | Neuroserpin: structure, function, physiology and pathology |
title_sort | neuroserpin: structure, function, physiology and pathology |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8558161/ https://www.ncbi.nlm.nih.gov/pubmed/34405255 http://dx.doi.org/10.1007/s00018-021-03907-6 |
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