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Hook2 contributes to aggresome formation

BACKGROUND: Aggresomes are pericentrosomal accumulations of misfolded proteins, chaperones and proteasomes. Their positioning near the centrosome, like that of other organelles, requires active, microtubule-dependent transport. Linker proteins that can associate with the motor protein dynein, organe...

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Autores principales: Szebenyi, Györgyi, Wigley, W Christian, Hall, Branden, Didier, Aaron, Yu, Michelle, Thomas, Philip, Krämer, Helmut
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1896156/
https://www.ncbi.nlm.nih.gov/pubmed/17540036
http://dx.doi.org/10.1186/1471-2121-8-19
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author Szebenyi, Györgyi
Wigley, W Christian
Hall, Branden
Didier, Aaron
Yu, Michelle
Thomas, Philip
Krämer, Helmut
author_facet Szebenyi, Györgyi
Wigley, W Christian
Hall, Branden
Didier, Aaron
Yu, Michelle
Thomas, Philip
Krämer, Helmut
author_sort Szebenyi, Györgyi
collection PubMed
description BACKGROUND: Aggresomes are pericentrosomal accumulations of misfolded proteins, chaperones and proteasomes. Their positioning near the centrosome, like that of other organelles, requires active, microtubule-dependent transport. Linker proteins that can associate with the motor protein dynein, organelles, and microtubules are thought to contribute to the active maintenance of the juxtanuclear localization of many membrane bound organelles and aggresomes. Hook proteins have been proposed to serve as adaptors for the association of cargos with dynein for transport on microtubules. Hook2 was shown to localize to the centrosome, bind centriolin, and contribute to centrosomal function. RESULTS: Here we show that overexpression of hook2 promotes the accumulation of the cystic fibrosis transmembrane regulator in aggresomes without altering its biochemical properties or its steady state level. A dominant negatively acting form of hook2 that lacks the centriolin binding C-terminal inhibits aggresome formation. CONCLUSION: We propose that hook2 contributes to the establishment and maintenance of the pericentrosomal localization of aggresomes by promoting the microtubule-based delivery of protein aggregates to pericentriolar aggresomes.
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spelling pubmed-18961562007-06-23 Hook2 contributes to aggresome formation Szebenyi, Györgyi Wigley, W Christian Hall, Branden Didier, Aaron Yu, Michelle Thomas, Philip Krämer, Helmut BMC Cell Biol Research Article BACKGROUND: Aggresomes are pericentrosomal accumulations of misfolded proteins, chaperones and proteasomes. Their positioning near the centrosome, like that of other organelles, requires active, microtubule-dependent transport. Linker proteins that can associate with the motor protein dynein, organelles, and microtubules are thought to contribute to the active maintenance of the juxtanuclear localization of many membrane bound organelles and aggresomes. Hook proteins have been proposed to serve as adaptors for the association of cargos with dynein for transport on microtubules. Hook2 was shown to localize to the centrosome, bind centriolin, and contribute to centrosomal function. RESULTS: Here we show that overexpression of hook2 promotes the accumulation of the cystic fibrosis transmembrane regulator in aggresomes without altering its biochemical properties or its steady state level. A dominant negatively acting form of hook2 that lacks the centriolin binding C-terminal inhibits aggresome formation. CONCLUSION: We propose that hook2 contributes to the establishment and maintenance of the pericentrosomal localization of aggresomes by promoting the microtubule-based delivery of protein aggregates to pericentriolar aggresomes. BioMed Central 2007-05-31 /pmc/articles/PMC1896156/ /pubmed/17540036 http://dx.doi.org/10.1186/1471-2121-8-19 Text en Copyright © 2007 Szebenyi et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Szebenyi, Györgyi
Wigley, W Christian
Hall, Branden
Didier, Aaron
Yu, Michelle
Thomas, Philip
Krämer, Helmut
Hook2 contributes to aggresome formation
title Hook2 contributes to aggresome formation
title_full Hook2 contributes to aggresome formation
title_fullStr Hook2 contributes to aggresome formation
title_full_unstemmed Hook2 contributes to aggresome formation
title_short Hook2 contributes to aggresome formation
title_sort hook2 contributes to aggresome formation
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1896156/
https://www.ncbi.nlm.nih.gov/pubmed/17540036
http://dx.doi.org/10.1186/1471-2121-8-19
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