mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex

Polycystic kidney disease (PKD) is a ciliopathy resulting from defective localization of membrane proteins such as PC-1 to the primary cilium, resulting in renal cysts, and is associated with another cystic genetic disease called tuberous sclerosis complex (TSC). Here the authors use kidney-specific...

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Autores principales:	Monika Pema, Luca Drusian, Marco Chiaravalli, Maddalena Castelli, Qin Yao, Sara Ricciardi, Stefan Somlo, Feng Qian, Stefano Biffo, Alessandra Boletta
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2016
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/5661208307ac486ca0832b838a77050f
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spelling	oai:doaj.org-article:5661208307ac486ca0832b838a77050f2021-12-02T15:33:49ZmTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex10.1038/ncomms107862041-1723https://doaj.org/article/5661208307ac486ca0832b838a77050f2016-03-01T00:00:00Zhttps://doi.org/10.1038/ncomms10786https://doaj.org/toc/2041-1723Polycystic kidney disease (PKD) is a ciliopathy resulting from defective localization of membrane proteins such as PC-1 to the primary cilium, resulting in renal cysts, and is associated with another cystic genetic disease called tuberous sclerosis complex (TSC). Here the authors use kidney-specific Tsc1 and Pkd1 mice to show that mTORC1 signalling inhibits PC-1 biogenesis as a potential mechanism of TSC/PKD contiguous gene syndrome.Monika PemaLuca DrusianMarco ChiaravalliMaddalena CastelliQin YaoSara RicciardiStefan SomloFeng QianStefano BiffoAlessandra BolettaNature PortfolioarticleScienceQENNature Communications, Vol 7, Iss 1, Pp 1-11 (2016)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Monika Pema Luca Drusian Marco Chiaravalli Maddalena Castelli Qin Yao Sara Ricciardi Stefan Somlo Feng Qian Stefano Biffo Alessandra Boletta mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
description	Polycystic kidney disease (PKD) is a ciliopathy resulting from defective localization of membrane proteins such as PC-1 to the primary cilium, resulting in renal cysts, and is associated with another cystic genetic disease called tuberous sclerosis complex (TSC). Here the authors use kidney-specific Tsc1 and Pkd1 mice to show that mTORC1 signalling inhibits PC-1 biogenesis as a potential mechanism of TSC/PKD contiguous gene syndrome.
format	article
author	Monika Pema Luca Drusian Marco Chiaravalli Maddalena Castelli Qin Yao Sara Ricciardi Stefan Somlo Feng Qian Stefano Biffo Alessandra Boletta
author_facet	Monika Pema Luca Drusian Marco Chiaravalli Maddalena Castelli Qin Yao Sara Ricciardi Stefan Somlo Feng Qian Stefano Biffo Alessandra Boletta
author_sort	Monika Pema
title	mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
title_short	mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
title_full	mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
title_fullStr	mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
title_full_unstemmed	mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
title_sort	mtorc1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex
publisher	Nature Portfolio
publishDate	2016
url	https://doaj.org/article/5661208307ac486ca0832b838a77050f
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mTORC1-mediated inhibition of polycystin-1 expression drives renal cyst formation in tuberous sclerosis complex

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