http://purl.uniprot.org/citations/17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/17848583 | http://www.w3.org/2000/01/rdf-schema#comment | "Patients with pulmonary alveolar proteinosis (PAP) display impaired surfactant clearance, foamy, lipid-filled alveolar macrophages, and increased cholesterol metabolites within the lung. Neutralizing autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF) are also present, resulting in virtual GM-CSF deficiency. We investigated ABCG1 and ABCA1 expression in alveolar macrophages of PAP patients and GM-CSF knockout (KO) mice, which exhibit PAP-like pulmonary pathology and increased pulmonary cholesterol. Alveolar macrophages from both sources displayed a striking similarity in transporter gene dysregulation, consisting of deficient ABCG1 accompanied by highly increased ABCA1. Peroxisome proliferator-activated receptor gamma (PPARgamma), a known regulator of both transporters, was deficient, as reported previously. In contrast, the liver X receptor alpha, which also upregulates both transporters, was highly increased. GM-CSF treatment increased ABCG1 expression in macrophages in vitro and in PAP patients in vivo. Overexpression of PPARgamma by lentivirus-PPARgamma transduction of primary alveolar macrophages, or activation by rosiglitazone, also increased ABCG1 expression. These results suggest that ABCG1 deficiency in PAP and GM-CSF KO alveolar macrophages is attributable to the absence of a GM-CSF-mediated PPARgamma pathway. These findings document the existence of ABCG1 deficiency in human lung disease and highlight a critical role for ABCG1 in surfactant homeostasis."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.org/dc/terms/identifier | "doi:10.1194/jlr.p700022-jlr200"xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Febbraio M."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Thomassen M.J."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Farver C.F."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Malur A."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Kavuru M.S."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Bonfield T.L."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Barna B.P."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Dalrymple H."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/author | "Malur A.G."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/date | "2007"xsd:gYear |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/name | "J Lipid Res"xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/pages | "2762-2768"xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/title | "ABCG1 is deficient in alveolar macrophages of GM-CSF knockout mice and patients with pulmonary alveolar proteinosis."xsd:string |
http://purl.uniprot.org/citations/17848583 | http://purl.uniprot.org/core/volume | "48"xsd:string |
http://purl.uniprot.org/citations/17848583 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/17848583 |
http://purl.uniprot.org/citations/17848583 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/17848583 |
http://purl.uniprot.org/uniprot/#_Q0VDW9-mappedCitation-17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17848583 |
http://purl.uniprot.org/uniprot/#_A0A494B9K1-mappedCitation-17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17848583 |
http://purl.uniprot.org/uniprot/#_A0A494B9N8-mappedCitation-17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17848583 |
http://purl.uniprot.org/uniprot/#_A0A494BA50-mappedCitation-17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17848583 |
http://purl.uniprot.org/uniprot/#_Q14AD9-mappedCitation-17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17848583 |
http://purl.uniprot.org/uniprot/#_Q64343-mappedCitation-17848583 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17848583 |