Matches in SemOpenAlex for { <https://semopenalex.org/work/W2283102561> ?p ?o ?g. }
Showing items 1 to 82 of
82
with 100 items per page.
- W2283102561 abstract "Pulmonary alveolar proteinosis (PAP) is a rare disease caused by the accumulation of surfactant proteins and lipids in the pulmonary alveoli resulting in respiratory distress. For almost all reported cases the underlying cause is unknown. Synthesis and secretion of surfactant in the lung is restricted to epithelial type 2 pneumocytes (T2 cells). However, subsequent clearance of surfactant from the hypophase is dependent upon both T2 cells and macrophages. Both T2 cells and macrophages express high levels of the sterol-transporter ATP Binding Cassette transporter G1 (ABCG1). Further, Abcg1-/- mice exhibit a severe pulmonary lipidosis, characterized by elevated levels of multiple lipid species and the presence of lipid-loaded macrophages and T2 cells containing abnormal lamellar bodies. Recent advances have implicated a role for specific granulocyte-macrophage colony stimulating factor (GM-CSF) autoantibodies and the innate immune system in the pathogenesis of PAP. We have recently demonstrated that the lungs of Abcg1-/- mice accumulate specific oxidized phospholipids and that this is associated with a local increase in innate immune natural antibody-secreting B-1 B cells and both local and circulating natural antibodies. Here we show that the lungs of mice containing ABCG1-deficient T2 (T2KO) cells and wild-type macrophages contain elevated levels of both phospholipids and esterified cholesterol. In addition, the T2KO cells in these mice accumulated lamellae bodies, unesterified cholesterol and exhibited abnormal gene expression. Collectively, our results suggest that ABCG1 is critical for normal surfactant metabolism and that the pulmonary expansion of B-1 B cells and the subsequent increase in natural antibodies in Abcg1-/- mice may provide a novel model in which to study the role of the innate immune system in PAP." @default.
- W2283102561 created "2016-06-24" @default.
- W2283102561 creator A5001830869 @default.
- W2283102561 creator A5005417593 @default.
- W2283102561 creator A5011059025 @default.
- W2283102561 creator A5031365548 @default.
- W2283102561 creator A5045659102 @default.
- W2283102561 creator A5051615442 @default.
- W2283102561 creator A5056419667 @default.
- W2283102561 creator A5059582644 @default.
- W2283102561 date "2015-05-01" @default.
- W2283102561 modified "2023-09-23" @default.
- W2283102561 title "Abstract 328: ABCG1 Has a Critical Role in Pulmonary Type 2 Cells and Surfactant Lipid Metabolism" @default.
- W2283102561 doi "https://doi.org/10.1161/atvb.35.suppl_1.328" @default.
- W2283102561 hasPublicationYear "2015" @default.
- W2283102561 type Work @default.
- W2283102561 sameAs 2283102561 @default.
- W2283102561 citedByCount "0" @default.
- W2283102561 crossrefType "journal-article" @default.
- W2283102561 hasAuthorship W2283102561A5001830869 @default.
- W2283102561 hasAuthorship W2283102561A5005417593 @default.
- W2283102561 hasAuthorship W2283102561A5011059025 @default.
- W2283102561 hasAuthorship W2283102561A5031365548 @default.
- W2283102561 hasAuthorship W2283102561A5045659102 @default.
- W2283102561 hasAuthorship W2283102561A5051615442 @default.
- W2283102561 hasAuthorship W2283102561A5056419667 @default.
- W2283102561 hasAuthorship W2283102561A5059582644 @default.
- W2283102561 hasConcept C104317684 @default.
- W2283102561 hasConcept C126322002 @default.
- W2283102561 hasConcept C136449434 @default.
- W2283102561 hasConcept C145156650 @default.
- W2283102561 hasConcept C149011108 @default.
- W2283102561 hasConcept C159654299 @default.
- W2283102561 hasConcept C185592680 @default.
- W2283102561 hasConcept C203014093 @default.
- W2283102561 hasConcept C2776330080 @default.
- W2283102561 hasConcept C2777704780 @default.
- W2283102561 hasConcept C2777714996 @default.
- W2283102561 hasConcept C2778972612 @default.
- W2283102561 hasConcept C4733338 @default.
- W2283102561 hasConcept C55493867 @default.
- W2283102561 hasConcept C58226133 @default.
- W2283102561 hasConcept C71924100 @default.
- W2283102561 hasConcept C86803240 @default.
- W2283102561 hasConcept C8891405 @default.
- W2283102561 hasConceptScore W2283102561C104317684 @default.
- W2283102561 hasConceptScore W2283102561C126322002 @default.
- W2283102561 hasConceptScore W2283102561C136449434 @default.
- W2283102561 hasConceptScore W2283102561C145156650 @default.
- W2283102561 hasConceptScore W2283102561C149011108 @default.
- W2283102561 hasConceptScore W2283102561C159654299 @default.
- W2283102561 hasConceptScore W2283102561C185592680 @default.
- W2283102561 hasConceptScore W2283102561C203014093 @default.
- W2283102561 hasConceptScore W2283102561C2776330080 @default.
- W2283102561 hasConceptScore W2283102561C2777704780 @default.
- W2283102561 hasConceptScore W2283102561C2777714996 @default.
- W2283102561 hasConceptScore W2283102561C2778972612 @default.
- W2283102561 hasConceptScore W2283102561C4733338 @default.
- W2283102561 hasConceptScore W2283102561C55493867 @default.
- W2283102561 hasConceptScore W2283102561C58226133 @default.
- W2283102561 hasConceptScore W2283102561C71924100 @default.
- W2283102561 hasConceptScore W2283102561C86803240 @default.
- W2283102561 hasConceptScore W2283102561C8891405 @default.
- W2283102561 hasIssue "suppl_1" @default.
- W2283102561 hasLocation W22831025611 @default.
- W2283102561 hasOpenAccess W2283102561 @default.
- W2283102561 hasPrimaryLocation W22831025611 @default.
- W2283102561 hasRelatedWork W2010361490 @default.
- W2283102561 hasRelatedWork W2027801782 @default.
- W2283102561 hasRelatedWork W2042813913 @default.
- W2283102561 hasRelatedWork W2131817432 @default.
- W2283102561 hasRelatedWork W2234353283 @default.
- W2283102561 hasRelatedWork W2283102561 @default.
- W2283102561 hasRelatedWork W2362032004 @default.
- W2283102561 hasRelatedWork W2591630293 @default.
- W2283102561 hasRelatedWork W278687238 @default.
- W2283102561 hasRelatedWork W999916044 @default.
- W2283102561 hasVolume "35" @default.
- W2283102561 isParatext "false" @default.
- W2283102561 isRetracted "false" @default.
- W2283102561 magId "2283102561" @default.
- W2283102561 workType "article" @default.