A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling.
Identifieur interne : 001627 ( Main/Exploration ); précédent : 001626; suivant : 001628A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling.
Auteurs : Markus Woischnik [Allemagne] ; Christiane Sparr ; Sun Ana Kern ; Tobias Thurm ; Andreas Hector ; Dominik Hartl ; Gerhard Liebisch ; Surafel Mulugeta ; Michael F. Beers ; Gerd Schmitz ; Matthias GrieseSource :
- BMC cell biology [ 1471-2121 ] ; 2010.
Descripteurs français
- KwdFr :
- Animaux, Azathioprine (usage thérapeutique), Calnexine (métabolisme), Calréticuline (métabolisme), Cellules épithéliales (métabolisme), Cyclophosphamide (usage thérapeutique), Humains, Hydroxychloroquine (usage thérapeutique), Lignée cellulaire, Liquide de lavage bronchoalvéolaire, Lymphocytes T CD4+ (immunologie), Mutation, Méthylprednisolone (usage thérapeutique), Phosphatidylcholines (métabolisme), Pneumopathies interstitielles (traitement médicamenteux), Protéine C associée au surfactant pulmonaire (analyse), Protéine C associée au surfactant pulmonaire (génétique), Protéine C associée au surfactant pulmonaire (métabolisme), Protéines du choc thermique HSP70 (métabolisme), Protéines du choc thermique HSP90 (métabolisme), Récepteurs CCR2 (métabolisme), Récepteurs à l'interleukine-8A (métabolisme), Souris, Substitution d'acide aminé, Transduction du signal.
- MESH :
- analyse : Protéine C associée au surfactant pulmonaire.
- génétique : Protéine C associée au surfactant pulmonaire.
- immunologie : Lymphocytes T CD4+.
- métabolisme : Calnexine, Calréticuline, Cellules épithéliales, Phosphatidylcholines, Protéine C associée au surfactant pulmonaire, Protéines du choc thermique HSP70, Protéines du choc thermique HSP90, Récepteurs CCR2, Récepteurs à l'interleukine-8A.
- traitement médicamenteux : Pneumopathies interstitielles.
- usage thérapeutique : Azathioprine, Cyclophosphamide, Hydroxychloroquine, Méthylprednisolone.
- Animaux, Humains, Lignée cellulaire, Liquide de lavage bronchoalvéolaire, Mutation, Souris, Substitution d'acide aminé, Transduction du signal.
English descriptors
- KwdEn :
- Amino Acid Substitution, Animals, Azathioprine (therapeutic use), Bronchoalveolar Lavage Fluid, CD4-Positive T-Lymphocytes (immunology), Calnexin (metabolism), Calreticulin (metabolism), Cell Line, Cyclophosphamide (therapeutic use), Epithelial Cells (metabolism), HSP70 Heat-Shock Proteins (metabolism), HSP90 Heat-Shock Proteins (metabolism), Humans, Hydroxychloroquine (therapeutic use), Lung Diseases, Interstitial (drug therapy), Methylprednisolone (therapeutic use), Mice, Mutation, Phosphatidylcholines (metabolism), Pulmonary Surfactant-Associated Protein C (analysis), Pulmonary Surfactant-Associated Protein C (genetics), Pulmonary Surfactant-Associated Protein C (metabolism), Receptors, CCR2 (metabolism), Receptors, Interleukin-8A (metabolism), Signal Transduction.
- MESH :
- chemical , analysis : Pulmonary Surfactant-Associated Protein C.
- chemical , genetics : Pulmonary Surfactant-Associated Protein C.
- chemical , metabolism : Calnexin, Calreticulin, HSP70 Heat-Shock Proteins, HSP90 Heat-Shock Proteins, Phosphatidylcholines, Pulmonary Surfactant-Associated Protein C, Receptors, CCR2, Receptors, Interleukin-8A.
- chemical , therapeutic use : Azathioprine, Cyclophosphamide, Hydroxychloroquine, Methylprednisolone.
- drug therapy : Lung Diseases, Interstitial.
- immunology : CD4-Positive T-Lymphocytes.
- metabolism : Epithelial Cells.
- Amino Acid Substitution, Animals, Bronchoalveolar Lavage Fluid, Cell Line, Humans, Mice, Mutation, Signal Transduction.
Abstract
Heterozygous mutations of SFTPC, the gene encoding surfactant protein C (SP-C), cause sporadic and familial interstitial lung disease (ILD) in children and adults. The most frequent SFTPC mutation in ILD patients leads to a threonine for isoleucine substitution at position 73 (I73T) of the SP-C preprotein (proSP-C), however little is known about the cellular consequences of SP-CI73T expression.
DOI: 10.1186/1471-2121-11-88
PubMed: 21092132
Affiliations:
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Le document en format XML
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Beers</name></author><author><name sortKey="Schmitz, Gerd" sort="Schmitz, Gerd" uniqKey="Schmitz G" first="Gerd" last="Schmitz">Gerd Schmitz</name></author><author><name sortKey="Griese, Matthias" sort="Griese, Matthias" uniqKey="Griese M" first="Matthias" last="Griese">Matthias Griese</name></author></analytic><series><title level="j">BMC cell biology</title><idno type="eISSN">1471-2121</idno><imprint><date when="2010" type="published">2010</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Amino Acid Substitution</term><term>Animals</term><term>Azathioprine (therapeutic use)</term><term>Bronchoalveolar Lavage Fluid</term><term>CD4-Positive T-Lymphocytes (immunology)</term><term>Calnexin (metabolism)</term><term>Calreticulin (metabolism)</term><term>Cell Line</term><term>Cyclophosphamide (therapeutic use)</term><term>Epithelial Cells (metabolism)</term><term>HSP70 Heat-Shock Proteins (metabolism)</term><term>HSP90 Heat-Shock Proteins (metabolism)</term><term>Humans</term><term>Hydroxychloroquine (therapeutic use)</term><term>Lung Diseases, Interstitial (drug therapy)</term><term>Methylprednisolone (therapeutic use)</term><term>Mice</term><term>Mutation</term><term>Phosphatidylcholines (metabolism)</term><term>Pulmonary Surfactant-Associated Protein C (analysis)</term><term>Pulmonary Surfactant-Associated Protein C (genetics)</term><term>Pulmonary Surfactant-Associated Protein C (metabolism)</term><term>Receptors, CCR2 (metabolism)</term><term>Receptors, Interleukin-8A (metabolism)</term><term>Signal Transduction</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Animaux</term><term>Azathioprine (usage thérapeutique)</term><term>Calnexine (métabolisme)</term><term>Calréticuline (métabolisme)</term><term>Cellules épithéliales (métabolisme)</term><term>Cyclophosphamide (usage thérapeutique)</term><term>Humains</term><term>Hydroxychloroquine (usage thérapeutique)</term><term>Lignée cellulaire</term><term>Liquide de lavage bronchoalvéolaire</term><term>Lymphocytes T CD4+ (immunologie)</term><term>Mutation</term><term>Méthylprednisolone (usage thérapeutique)</term><term>Phosphatidylcholines (métabolisme)</term><term>Pneumopathies interstitielles (traitement médicamenteux)</term><term>Protéine C associée au surfactant pulmonaire (analyse)</term><term>Protéine C associée au surfactant pulmonaire (génétique)</term><term>Protéine C associée au surfactant pulmonaire (métabolisme)</term><term>Protéines du choc thermique HSP70 (métabolisme)</term><term>Protéines du choc thermique HSP90 (métabolisme)</term><term>Récepteurs CCR2 (métabolisme)</term><term>Récepteurs à l'interleukine-8A (métabolisme)</term><term>Souris</term><term>Substitution d'acide aminé</term><term>Transduction du signal</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Pulmonary Surfactant-Associated Protein C</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Pulmonary Surfactant-Associated Protein C</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Calnexin</term><term>Calreticulin</term><term>HSP70 Heat-Shock Proteins</term><term>HSP90 Heat-Shock Proteins</term><term>Phosphatidylcholines</term><term>Pulmonary Surfactant-Associated Protein C</term><term>Receptors, CCR2</term><term>Receptors, Interleukin-8A</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Azathioprine</term><term>Cyclophosphamide</term><term>Hydroxychloroquine</term><term>Methylprednisolone</term></keywords><keywords scheme="MESH" qualifier="analyse" xml:lang="fr"><term>Protéine C associée au surfactant pulmonaire</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Lung Diseases, Interstitial</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Protéine C associée au surfactant pulmonaire</term></keywords><keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Lymphocytes T CD4+</term></keywords><keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>CD4-Positive T-Lymphocytes</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Epithelial Cells</term></keywords><keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Calnexine</term><term>Calréticuline</term><term>Cellules épithéliales</term><term>Phosphatidylcholines</term><term>Protéine C associée au surfactant pulmonaire</term><term>Protéines du choc thermique HSP70</term><term>Protéines du choc thermique HSP90</term><term>Récepteurs CCR2</term><term>Récepteurs à l'interleukine-8A</term></keywords><keywords scheme="MESH" qualifier="traitement médicamenteux" xml:lang="fr"><term>Pneumopathies interstitielles</term></keywords><keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Azathioprine</term><term>Cyclophosphamide</term><term>Hydroxychloroquine</term><term>Méthylprednisolone</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Amino Acid Substitution</term><term>Animals</term><term>Bronchoalveolar Lavage Fluid</term><term>Cell Line</term><term>Humans</term><term>Mice</term><term>Mutation</term><term>Signal Transduction</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Animaux</term><term>Humains</term><term>Lignée cellulaire</term><term>Liquide de lavage bronchoalvéolaire</term><term>Mutation</term><term>Souris</term><term>Substitution d'acide aminé</term><term>Transduction du signal</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Heterozygous mutations of SFTPC, the gene encoding surfactant protein C (SP-C), cause sporadic and familial interstitial lung disease (ILD) in children and adults. The most frequent SFTPC mutation in ILD patients leads to a threonine for isoleucine substitution at position 73 (I73T) of the SP-C preprotein (proSP-C), however little is known about the cellular consequences of SP-CI73T expression.</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><orgName><li>Université Louis-et-Maximilien de Munich</li></orgName></list><tree><noCountry><name sortKey="Beers, Michael F" sort="Beers, Michael F" uniqKey="Beers M" first="Michael F" last="Beers">Michael F. Beers</name><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="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="Mulugeta, Surafel" sort="Mulugeta, Surafel" uniqKey="Mulugeta S" first="Surafel" last="Mulugeta">Surafel Mulugeta</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></noCountry><country name="Allemagne"><region name="Bavière"><name sortKey="Woischnik, Markus" sort="Woischnik, Markus" uniqKey="Woischnik M" first="Markus" last="Woischnik">Markus Woischnik</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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