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A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling

Woischnik, Markus and Sparr, Christiane and Kern, Sunčana and Thurm, Tobias and Hector, Andreas and Hartl, Dominik and Liebisch, Gerhard and Mulugeta, Surafel and Beers, Michael F. and Schmitz, Gerd and Griese, Matthias (2010) A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling. BMC cell biology 11, p. 88.

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Abstract

We show that I73T mutation leads to impaired processing of proSP-C in alveolar type II cells, alters their stress tolerance and surfactant lipid composition, and activates cells of the immune system. In addition, we show that some of the mentioned cellular aspects behind the disease can be modulated by application of pharmaceutical drugs commonly applied in the ILD ...

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Item Type:Article
Date:2010
Institutions:Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin
Identification Number:
ValueType
21092132PubMed ID
10.1186/1471-2121-11-88DOI
Classification:
NotationType
Amino Acid SubstitutionMESH
AnimalsMESH
Azathioprine/therapeutic useMESH
Bronchoalveolar Lavage FluidMESH
CD4-Positive T-Lymphocytes/immunologyMESH
Calnexin/metabolismMESH
Calreticulin/metabolismMESH
Cell LineMESH
Cyclophosphamide/therapeutic useMESH
Epithelial Cells/metabolismMESH
HSP70 Heat-Shock Proteins/metabolismMESH
HSP90 Heat-Shock Proteins/metabolismMESH
HumansMESH
Hydroxychloroquine/therapeutic useMESH
Lung Diseases, Interstitial/drug therapyMESH
Methylprednisolone/therapeutic useMESH
MiceMESH
MutationMESH
Phosphatidylcholines/metabolismMESH
Pulmonary Surfactant-Associated Protein C/metabolismMESH
Receptors, CCR2/metabolismMESH
Receptors, Interleukin-8A/metabolismMESH
Signal TransductionMESH
Subjects:600 Technology > 610 Medical sciences Medicine
Status:Published
Refereed:Yes, this version has been refereed
Created at the University of Regensburg:Unknown
Owner: Universitätsbibliothek Regensburg
Deposited On:26 Sep 2011 09:47
Last Modified:13 Mar 2014 18:02
Item ID:22190
Owner Only: item control page
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