Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations

Mutations in the granulin gene are associated with frontotemporal lobe dementia (FTLD) and a lysosomal storage disease. The authors show that reduced progranulin levels leads to impaired neuronal uptake and lysosomal delivery of prosaposin, and that decreased prosaposin expression in mice leads to F...

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Autores principales: Xiaolai Zhou, Lirong Sun, Oliver Bracko, Ji Whae Choi, Yan Jia, Alissa L. Nana, Owen Adam Brady, Jean C. Cruz Hernandez, Nozomi Nishimura, William W. Seeley, Fenghua Hu
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Lenguaje:EN
Publicado: Nature Portfolio 2017
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Acceso en línea:https://doaj.org/article/41d78f62c0c2475397bd2fa199e6bc5b
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spelling oai:doaj.org-article:41d78f62c0c2475397bd2fa199e6bc5b2021-12-02T17:06:28ZImpaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations10.1038/ncomms152772041-1723https://doaj.org/article/41d78f62c0c2475397bd2fa199e6bc5b2017-05-01T00:00:00Zhttps://doi.org/10.1038/ncomms15277https://doaj.org/toc/2041-1723Mutations in the granulin gene are associated with frontotemporal lobe dementia (FTLD) and a lysosomal storage disease. The authors show that reduced progranulin levels leads to impaired neuronal uptake and lysosomal delivery of prosaposin, and that decreased prosaposin expression in mice leads to FTLD-like behaviour.Xiaolai ZhouLirong SunOliver BrackoJi Whae ChoiYan JiaAlissa L. NanaOwen Adam BradyJean C. Cruz HernandezNozomi NishimuraWilliam W. SeeleyFenghua HuNature PortfolioarticleScienceQENNature Communications, Vol 8, Iss 1, Pp 1-14 (2017)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Xiaolai Zhou
Lirong Sun
Oliver Bracko
Ji Whae Choi
Yan Jia
Alissa L. Nana
Owen Adam Brady
Jean C. Cruz Hernandez
Nozomi Nishimura
William W. Seeley
Fenghua Hu
Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
description Mutations in the granulin gene are associated with frontotemporal lobe dementia (FTLD) and a lysosomal storage disease. The authors show that reduced progranulin levels leads to impaired neuronal uptake and lysosomal delivery of prosaposin, and that decreased prosaposin expression in mice leads to FTLD-like behaviour.
format article
author Xiaolai Zhou
Lirong Sun
Oliver Bracko
Ji Whae Choi
Yan Jia
Alissa L. Nana
Owen Adam Brady
Jean C. Cruz Hernandez
Nozomi Nishimura
William W. Seeley
Fenghua Hu
author_facet Xiaolai Zhou
Lirong Sun
Oliver Bracko
Ji Whae Choi
Yan Jia
Alissa L. Nana
Owen Adam Brady
Jean C. Cruz Hernandez
Nozomi Nishimura
William W. Seeley
Fenghua Hu
author_sort Xiaolai Zhou
title Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
title_short Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
title_full Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
title_fullStr Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
title_full_unstemmed Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
title_sort impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations
publisher Nature Portfolio
publishDate 2017
url https://doaj.org/article/41d78f62c0c2475397bd2fa199e6bc5b
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