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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 therapy.

Item Type:

Article

Institutions:

Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin

Identification Number:

Value	Type
21092132	PubMed ID
10.1186/1471-2121-11-88	DOI

Classification:

Notation	Type
Amino Acid Substitution	MESH
Animals	MESH
Azathioprine/therapeutic use	MESH
Bronchoalveolar Lavage Fluid	MESH
CD4-Positive T-Lymphocytes/immunology	MESH
Calnexin/metabolism	MESH
Calreticulin/metabolism	MESH
Cell Line	MESH
Cyclophosphamide/therapeutic use	MESH
Epithelial Cells/metabolism	MESH
HSP70 Heat-Shock Proteins/metabolism	MESH
HSP90 Heat-Shock Proteins/metabolism	MESH
Humans	MESH
Hydroxychloroquine/therapeutic use	MESH
Lung Diseases, Interstitial/drug therapy	MESH
Methylprednisolone/therapeutic use	MESH
Mice	MESH
Mutation	MESH
Phosphatidylcholines/metabolism	MESH
Pulmonary Surfactant-Associated Protein C/metabolism	MESH
Receptors, CCR2/metabolism	MESH
Receptors, Interleukin-8A/metabolism	MESH
Signal Transduction	MESH