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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...

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Detalles Bibliográficos
Autores principales: Sun, Li, Eriksen, Jason L
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bentham Science Publishers 2011
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.
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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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