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Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia
Progranulin is a widely expressed protein that is involved in the regulation of multiple biological processes, including embryogenesis, host defense, and wound repair. In the central nervous system, progranulin is constitutively expressed at modest levels in neurons and microglia, but shows dramatic...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Bentham Science Publishers
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3263457/ https://www.ncbi.nlm.nih.gov/pubmed/22654721 http://dx.doi.org/10.2174/157015911798376361 |
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author | Sun, Li Eriksen, Jason L |
author_facet | Sun, Li Eriksen, Jason L |
author_sort | Sun, Li |
collection | PubMed |
description | Progranulin is a widely expressed protein that is involved in the regulation of multiple biological processes, including embryogenesis, host defense, and wound repair. In the central nervous system, progranulin is constitutively expressed at modest levels in neurons and microglia, but shows dramatic microglial immunoreactivity in degenerative diseases that exhibit prominent neuroinflammation. In addition to the role that PGRN plays in the periphery, its expression is of critical importance in brain health, as demonstrated by recent discovery that progranulin haploinsufficiency results in familial frontotemporal lobar degeneration. Since progranulin deficiency was first described, there has been an intense ongoing effort to decipher the mysterious role that this protein plays in dementia. This review provides an update on our understanding of the possible neuronal function and discusses the challenging problems related to progranulin expression within genetics, cell biology, and neurodegeneration. |
format | Online Article Text |
id | pubmed-3263457 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Bentham Science Publishers |
record_format | MEDLINE/PubMed |
spelling | pubmed-32634572012-06-01 Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia Sun, Li Eriksen, Jason L Curr Neuropharmacol Article Progranulin is a widely expressed protein that is involved in the regulation of multiple biological processes, including embryogenesis, host defense, and wound repair. In the central nervous system, progranulin is constitutively expressed at modest levels in neurons and microglia, but shows dramatic microglial immunoreactivity in degenerative diseases that exhibit prominent neuroinflammation. In addition to the role that PGRN plays in the periphery, its expression is of critical importance in brain health, as demonstrated by recent discovery that progranulin haploinsufficiency results in familial frontotemporal lobar degeneration. Since progranulin deficiency was first described, there has been an intense ongoing effort to decipher the mysterious role that this protein plays in dementia. This review provides an update on our understanding of the possible neuronal function and discusses the challenging problems related to progranulin expression within genetics, cell biology, and neurodegeneration. Bentham Science Publishers 2011-12 /pmc/articles/PMC3263457/ /pubmed/22654721 http://dx.doi.org/10.2174/157015911798376361 Text en ©2011 Bentham Science Publishers http://creativecommons.org/licenses/by/2.5/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.5/), which permits unrestrictive use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Article Sun, Li Eriksen, Jason L Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia |
title | Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia |
title_full | Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia |
title_fullStr | Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia |
title_full_unstemmed | Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia |
title_short | Recent Insights into the Involvement of Progranulin in Frontotemporal Dementia |
title_sort | recent insights into the involvement of progranulin in frontotemporal dementia |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3263457/ https://www.ncbi.nlm.nih.gov/pubmed/22654721 http://dx.doi.org/10.2174/157015911798376361 |
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