The surfactant protein C mutation A116D alters cellular processing, stress tolerance, surfactant lipid composition, and immune cell activation.
Identifieur interne : 001296 ( Main/Exploration ); précédent : 001295; suivant : 001297The surfactant protein C mutation A116D alters cellular processing, stress tolerance, surfactant lipid composition, and immune cell activation.
Auteurs : Ralf Zarbock [Allemagne] ; Markus Woischnik ; Christiane Sparr ; Tobias Thurm ; Sun Ana Kern ; Eva Kaltenborn ; Andreas Hector ; Dominik Hartl ; Gerhard Liebisch ; Gerd Schmitz ; Matthias GrieseSource :
- BMC pulmonary medicine [ 1471-2466 ] ; 2012.
Descripteurs français
- KwdFr :
- Alvéoles pulmonaires (), Alvéoles pulmonaires (immunologie), Alvéoles pulmonaires (métabolisme), Animaux, Azathioprine (pharmacologie), Cellules épithéliales (), Cellules épithéliales (immunologie), Cellules épithéliales (métabolisme), Chaperons moléculaires (génétique), Chaperons moléculaires (métabolisme), Cyclophosphamide (pharmacologie), Expression des gènes, Humains, Hydroxychloroquine (pharmacologie), Lignée cellulaire, Mutation, Méthylprednisolone (pharmacologie), Phospholipides (analyse), Phospholipides (génétique), Phospholipides (métabolisme), Pneumopathies interstitielles (génétique), Pneumopathies interstitielles (immunologie), Protéine C associée au surfactant pulmonaire (génétique), Protéine C associée au surfactant pulmonaire (immunologie), Souris, Surfactants pulmonaires (métabolisme).
- MESH :
- analyse : Phospholipides.
- génétique : Chaperons moléculaires, Phospholipides, Pneumopathies interstitielles, Protéine C associée au surfactant pulmonaire.
- immunologie : Alvéoles pulmonaires, Cellules épithéliales, Pneumopathies interstitielles, Protéine C associée au surfactant pulmonaire.
- métabolisme : Alvéoles pulmonaires, Cellules épithéliales, Chaperons moléculaires, Phospholipides, Surfactants pulmonaires.
- pharmacologie : Azathioprine, Cyclophosphamide, Hydroxychloroquine, Méthylprednisolone.
- Alvéoles pulmonaires, Animaux, Cellules épithéliales, Expression des gènes, Humains, Lignée cellulaire, Mutation, Souris.
English descriptors
- KwdEn :
- Animals, Azathioprine (pharmacology), Cell Line, Cyclophosphamide (pharmacology), Epithelial Cells (drug effects), Epithelial Cells (immunology), Epithelial Cells (metabolism), Gene Expression, Humans, Hydroxychloroquine (pharmacology), Lung Diseases, Interstitial (genetics), Lung Diseases, Interstitial (immunology), Methylprednisolone (pharmacology), Mice, Molecular Chaperones (genetics), Molecular Chaperones (metabolism), Mutation, Phospholipids (analysis), Phospholipids (genetics), Phospholipids (metabolism), Pulmonary Alveoli (drug effects), Pulmonary Alveoli (immunology), Pulmonary Alveoli (metabolism), Pulmonary Surfactant-Associated Protein C (genetics), Pulmonary Surfactant-Associated Protein C (immunology), Pulmonary Surfactants (metabolism).
- MESH :
- chemical , analysis : Phospholipids.
- chemical , genetics : Molecular Chaperones, Phospholipids, Pulmonary Surfactant-Associated Protein C.
- chemical , immunology : Pulmonary Surfactant-Associated Protein C.
- chemical , metabolism : Molecular Chaperones, Phospholipids, Pulmonary Surfactants.
- chemical , pharmacology : Azathioprine, Cyclophosphamide, Hydroxychloroquine, Methylprednisolone.
- drug effects : Epithelial Cells, Pulmonary Alveoli.
- genetics : Lung Diseases, Interstitial.
- immunology : Epithelial Cells, Lung Diseases, Interstitial, Pulmonary Alveoli.
- metabolism : Epithelial Cells, Pulmonary Alveoli.
- Animals, Cell Line, Gene Expression, Humans, Mice, Mutation.
Abstract
Surfactant protein C (SP-C) is important for the function of pulmonary surfactant. Heterozygous mutations in SFTPC, the gene encoding SP-C, cause sporadic and familial interstitial lung disease (ILD) in children and adults. Mutations mapping to the BRICHOS domain located within the SP-C proprotein result in perinuclear aggregation of the proprotein. In this study, we investigated the effects of the mutation A116D in the BRICHOS domain of SP-C on cellular homeostasis. We also evaluated the ability of drugs currently used in ILD therapy to counteract these effects.
DOI: 10.1186/1471-2466-12-15
PubMed: 22458263
Affiliations:
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au surfactant pulmonaire (immunologie)</term><term>Souris</term><term>Surfactants pulmonaires (métabolisme)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Phospholipids</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Molecular Chaperones</term><term>Phospholipids</term><term>Pulmonary Surfactant-Associated Protein C</term></keywords><keywords scheme="MESH" type="chemical" qualifier="immunology" xml:lang="en"><term>Pulmonary Surfactant-Associated Protein C</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Molecular Chaperones</term><term>Phospholipids</term><term>Pulmonary Surfactants</term></keywords><keywords scheme="MESH" type="chemical" qualifier="pharmacology" xml:lang="en"><term>Azathioprine</term><term>Cyclophosphamide</term><term>Hydroxychloroquine</term><term>Methylprednisolone</term></keywords><keywords scheme="MESH" 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cellulaire</term><term>Mutation</term><term>Souris</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Surfactant protein C (SP-C) is important for the function of pulmonary surfactant. Heterozygous mutations in SFTPC, the gene encoding SP-C, cause sporadic and familial interstitial lung disease (ILD) in children and adults. Mutations mapping to the BRICHOS domain located within the SP-C proprotein result in perinuclear aggregation of the proprotein. In this study, we investigated the effects of the mutation A116D in the BRICHOS domain of SP-C on cellular homeostasis. We also evaluated the ability of drugs currently used in ILD therapy to counteract these effects.</div></front></TEI><affiliations><list><country><li>Allemagne</li></country><region><li>Bavière</li><li>District de Haute-Bavière</li></region><settlement><li>Munich</li></settlement></list><tree><noCountry><name sortKey="Griese, Matthias" sort="Griese, Matthias" uniqKey="Griese M" first="Matthias" last="Griese">Matthias Griese</name><name sortKey="Hartl, Dominik" sort="Hartl, Dominik" uniqKey="Hartl D" first="Dominik" last="Hartl">Dominik Hartl</name><name sortKey="Hector, Andreas" sort="Hector, Andreas" uniqKey="Hector A" first="Andreas" last="Hector">Andreas Hector</name><name sortKey="Kaltenborn, Eva" sort="Kaltenborn, Eva" uniqKey="Kaltenborn E" first="Eva" last="Kaltenborn">Eva Kaltenborn</name><name sortKey="Kern, Sun Ana" sort="Kern, Sun Ana" uniqKey="Kern S" first="Sun Ana" last="Kern">Sun Ana Kern</name><name sortKey="Liebisch, Gerhard" sort="Liebisch, Gerhard" uniqKey="Liebisch G" first="Gerhard" last="Liebisch">Gerhard Liebisch</name><name sortKey="Schmitz, Gerd" sort="Schmitz, Gerd" uniqKey="Schmitz G" first="Gerd" last="Schmitz">Gerd Schmitz</name><name sortKey="Sparr, Christiane" sort="Sparr, Christiane" uniqKey="Sparr C" first="Christiane" last="Sparr">Christiane Sparr</name><name sortKey="Thurm, Tobias" sort="Thurm, Tobias" uniqKey="Thurm T" first="Tobias" last="Thurm">Tobias Thurm</name><name sortKey="Woischnik, Markus" sort="Woischnik, Markus" uniqKey="Woischnik M" first="Markus" last="Woischnik">Markus Woischnik</name></noCountry><country name="Allemagne"><region name="Bavière"><name sortKey="Zarbock, Ralf" sort="Zarbock, Ralf" uniqKey="Zarbock R" first="Ralf" last="Zarbock">Ralf Zarbock</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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