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High-resolution str...
High-resolution structure of a BRICHOS domain and its implications for anti-amyloid chaperone activity on lung surfactant protein C
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- Willander, Hanna (author)
- Karolinska Institutet,Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för anatomi, fysiologi och biokemi,Department of Anatomy, Physiology and Biochemistry (AFB)
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- Askarieh, Glareh (author)
- Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för molekylärbiologi,Department of Molecular Biology
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- Landreh, Michael (author)
- Karolinska Institutet
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- Westermark, Per (author)
- Uppsala universitet,Molekylär och morfologisk patologi,Per Westermark
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- Nordling, Kerstin (author)
- Karolinska Institutet,Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för anatomi, fysiologi och biokemi,Department of Anatomy, Physiology and Biochemistry (AFB)
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- Keränen, Henrik (author)
- Uppsala universitet,Beräknings- och systembiologi
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- Hermansson, Erik (author)
- Karolinska Institutet,Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för anatomi, fysiologi och biokemi,Department of Anatomy, Physiology and Biochemistry (AFB)
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Hamvas, Aaron (author)
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Nogee, Lawrence M. (author)
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- Bergman, Tomas (author)
- Karolinska Institutet
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Saenz, Alejandra (author)
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Casals, Cristina (author)
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- Åqvist, Johan (author)
- Uppsala universitet,Beräknings- och systembiologi
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- Jörnvall, Hans (author)
- Karolinska Institutet
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- Berglund, Helena (author)
- Karolinska Institutet
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- Presto, Jenny (author)
- Karolinska Institutet,Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för anatomi, fysiologi och biokemi,Department of Anatomy, Physiology and Biochemistry (AFB),Department of Anatomy, Physiology, and Biochemistry, Swedish University of Agricultural Sciences, S-751 24 Uppsala, Sweden
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- Knight, Stefan D. (author)
- Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för molekylärbiologi,Department of Molecular Biology,Department of Molecular Biology, Swedish University of Agricultural Sciences, S-751 24 Uppsala, Sweden;
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- Johansson, Jan (author)
- Karolinska Institutet,Swedish University of Agricultural Sciences,Sveriges lantbruksuniversitet,Institutionen för anatomi, fysiologi och biokemi,Department of Anatomy, Physiology and Biochemistry (AFB)
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(creator_code:org_t)
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- 2012-02-02
- 2012
- English.
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In: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424 .- 1091-6490. ; 109:7, s. 2325-2329
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Abstract
Subject headings
Close
- BRICHOS domains are encoded in >30 human genes, which are associated with cancer, neurodegeneration, and interstitial lung disease (ILD). The BRICHOS domain from lung surfactant protein C proprotein (proSP-C) is required for membrane insertion of SP-C and has anti-amyloid activity in vitro. Here, we report the 2.1 angstrom crystal structure of the human proSP-C BRICHOS domain, which, together with molecular dynamics simulations and hydrogen-deuterium exchange mass spectrometry, reveals how BRICHOS domains may mediate chaperone activity. Observation of amyloid deposits composed of mature SP-C in lung tissue samples from ILD patients with mutations in the BRICHOS domain or in its peptide-binding linker region supports the in vivo relevance of the proposed mechanism. The results indicate that ILD mutations interfering with proSP-C BRICHOS activity cause amyloid disease secondary to intramolecular chaperone malfunction.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Cell- och molekylärbiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Cell and Molecular Biology (hsv//eng)
Keyword
- interstitial lung disease
- SFTPC mutations
- beta-sheet aggregates
- transmembrane segment
- discordant helix
Publication and Content Type
- ref (subject category)
- art (subject category)
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To the university's database
- By the author/editor
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Willander, Hanna
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Askarieh, Glareh
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Landreh, Michael
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Westermark, Per
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Nordling, Kersti ...
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Keränen, Henrik
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Hermansson, Erik
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Hamvas, Aaron
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Nogee, Lawrence ...
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Bergman, Tomas
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Saenz, Alejandra
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Casals, Cristina
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Åqvist, Johan
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Jörnvall, Hans
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Berglund, Helena
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Presto, Jenny
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Knight, Stefan D ...
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Johansson, Jan
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Cell and Molecul ...
- Articles in the publication
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Proceedings of t ...
- By the university
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Uppsala University
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Swedish University of Agricultural Sciences
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Karolinska Institutet