C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
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Full title
Author / Creator
Zhang, Yong-Jie , Gendron, Tania F , Grima, Jonathan C , Sasaguri, Hiroki , Jansen-West, Karen , Xu, Ya-Fei , Katzman, Rebecca B , Gass, Jennifer , Murray, Melissa E , Shinohara, Mitsuru , Lin, Wen-Lang , Garrett, Aliesha , Stankowski, Jeannette N , Daughrity, Lillian , Tong, Jimei , Perkerson, Emilie A , Yue, Mei , Chew, Jeannie , Castanedes-Casey, Monica , Kurti, Aishe , Wang, Zizhao S , Liesinger, Amanda M , Baker, Jeremy D , Jiang, Jie , Lagier-Tourenne, Clotilde , Edbauer, Dieter , Cleveland, Don W , Rademakers, Rosa , Boylan, Kevin B , Bu, Guojun , Link, Christopher D , Dickey, Chad A , Rothstein, Jeffrey D , Dickson, Dennis W , Fryer, John D and Petrucelli, Leonard
Publisher
New York: Nature Publishing Group US
Journal title
Language
English
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Publication information
Publisher
New York: Nature Publishing Group US
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More information
Scope and Contents
Contents
Zhang
et al
. show that the poly(GA) proteins produced in patients with
C9ORF72
repeat expansions cause neurodegeneration and behavioral abnormalities when expressed in mice. The emergence of these phenotypes requires poly(GA) aggregation, and poly(GA) inclusions sequester HR23 proteins involved in proteasomal degradation, as well as pr...
Alternative Titles
Full title
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
Authors, Artists and Contributors
Author / Creator
Gendron, Tania F
Grima, Jonathan C
Sasaguri, Hiroki
Jansen-West, Karen
Xu, Ya-Fei
Katzman, Rebecca B
Gass, Jennifer
Murray, Melissa E
Shinohara, Mitsuru
Lin, Wen-Lang
Garrett, Aliesha
Stankowski, Jeannette N
Daughrity, Lillian
Tong, Jimei
Perkerson, Emilie A
Yue, Mei
Chew, Jeannie
Castanedes-Casey, Monica
Kurti, Aishe
Wang, Zizhao S
Liesinger, Amanda M
Baker, Jeremy D
Jiang, Jie
Lagier-Tourenne, Clotilde
Edbauer, Dieter
Cleveland, Don W
Rademakers, Rosa
Boylan, Kevin B
Bu, Guojun
Link, Christopher D
Dickey, Chad A
Rothstein, Jeffrey D
Dickson, Dennis W
Fryer, John D
Petrucelli, Leonard
Identifiers
Primary Identifiers
Record Identifier
TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5138863
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5138863
Other Identifiers
ISSN
1097-6256
E-ISSN
1546-1726
DOI
10.1038/nn.4272
