CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL

Low density lipoprotein receptor (LDLR) is crucial for cholesterol homeostasis. Here, the authors show that components of the CCC-protein complex, CCDC22 and COMMD1, facilitate the endosomal sorting of LDLR and that mutations in these genes cause hypercholesterolemia in dogs and mice, providing new...

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Autores principales: Paulina Bartuzi, Daniel D. Billadeau, Robert Favier, Shunxing Rong, Daphne Dekker, Alina Fedoseienko, Hille Fieten, Melinde Wijers, Johannes H. Levels, Nicolette Huijkman, Niels Kloosterhuis, Henk van der Molen, Gemma Brufau, Albert K. Groen, Alison M. Elliott, Jan Albert Kuivenhoven, Barbara Plecko, Gernot Grangl, Julie McGaughran, Jay D. Horton, Ezra Burstein, Marten H. Hofker, Bart van de Sluis
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Lenguaje:EN
Publicado: Nature Portfolio 2016
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Acceso en línea:https://doaj.org/article/8e60ef48969c43c189785a273159992f
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spelling oai:doaj.org-article:8e60ef48969c43c189785a273159992f2021-12-02T16:57:40ZCCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL10.1038/ncomms109612041-1723https://doaj.org/article/8e60ef48969c43c189785a273159992f2016-03-01T00:00:00Zhttps://doi.org/10.1038/ncomms10961https://doaj.org/toc/2041-1723Low density lipoprotein receptor (LDLR) is crucial for cholesterol homeostasis. Here, the authors show that components of the CCC-protein complex, CCDC22 and COMMD1, facilitate the endosomal sorting of LDLR and that mutations in these genes cause hypercholesterolemia in dogs and mice, providing new insights into regulation of cholesterol homeostasis.Paulina BartuziDaniel D. BilladeauRobert FavierShunxing RongDaphne DekkerAlina FedoseienkoHille FietenMelinde WijersJohannes H. LevelsNicolette HuijkmanNiels KloosterhuisHenk van der MolenGemma BrufauAlbert K. GroenAlison M. ElliottJan Albert KuivenhovenBarbara PleckoGernot GranglJulie McGaughranJay D. HortonEzra BursteinMarten H. HofkerBart van de SluisNature PortfolioarticleScienceQENNature Communications, Vol 7, Iss 1, Pp 1-11 (2016)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Paulina Bartuzi
Daniel D. Billadeau
Robert Favier
Shunxing Rong
Daphne Dekker
Alina Fedoseienko
Hille Fieten
Melinde Wijers
Johannes H. Levels
Nicolette Huijkman
Niels Kloosterhuis
Henk van der Molen
Gemma Brufau
Albert K. Groen
Alison M. Elliott
Jan Albert Kuivenhoven
Barbara Plecko
Gernot Grangl
Julie McGaughran
Jay D. Horton
Ezra Burstein
Marten H. Hofker
Bart van de Sluis
CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL
description Low density lipoprotein receptor (LDLR) is crucial for cholesterol homeostasis. Here, the authors show that components of the CCC-protein complex, CCDC22 and COMMD1, facilitate the endosomal sorting of LDLR and that mutations in these genes cause hypercholesterolemia in dogs and mice, providing new insights into regulation of cholesterol homeostasis.
format article
author Paulina Bartuzi
Daniel D. Billadeau
Robert Favier
Shunxing Rong
Daphne Dekker
Alina Fedoseienko
Hille Fieten
Melinde Wijers
Johannes H. Levels
Nicolette Huijkman
Niels Kloosterhuis
Henk van der Molen
Gemma Brufau
Albert K. Groen
Alison M. Elliott
Jan Albert Kuivenhoven
Barbara Plecko
Gernot Grangl
Julie McGaughran
Jay D. Horton
Ezra Burstein
Marten H. Hofker
Bart van de Sluis
author_facet Paulina Bartuzi
Daniel D. Billadeau
Robert Favier
Shunxing Rong
Daphne Dekker
Alina Fedoseienko
Hille Fieten
Melinde Wijers
Johannes H. Levels
Nicolette Huijkman
Niels Kloosterhuis
Henk van der Molen
Gemma Brufau
Albert K. Groen
Alison M. Elliott
Jan Albert Kuivenhoven
Barbara Plecko
Gernot Grangl
Julie McGaughran
Jay D. Horton
Ezra Burstein
Marten H. Hofker
Bart van de Sluis
author_sort Paulina Bartuzi
title CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL
title_short CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL
title_full CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL
title_fullStr CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL
title_full_unstemmed CCC- and WASH-mediated endosomal sorting of LDLR is required for normal clearance of circulating LDL
title_sort ccc- and wash-mediated endosomal sorting of ldlr is required for normal clearance of circulating ldl
publisher Nature Portfolio
publishDate 2016
url https://doaj.org/article/8e60ef48969c43c189785a273159992f
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