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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Nature Portfolio
2017
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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) |
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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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