Abstract 551: Loss of ABCG1 Specifically in Pulmonary Type 2 Cells Results in Impaired Surfactant Lipid Metabolism

• DOI: 10.1161/atvb.36.suppl_1.551
• Publication Date: 2021-06-21T16:58:35Z
• Authors: Thomas Q de Aguia...; David J Merriott; Joan Cheng; Angela Cheng; Angel Baldan; David A Ford; Christopher N Gou...; Elizabeth J Tarling
• Keywords: Lamellar granule; Pulmonary alveolar proteinosis; ABCG1

Cardiovascular disease (CVD) is the leading cause of death in United States, and patients with pulmonary are at least 2-fold more likely to develop cardiovascular complications. Whole-body cholesterol balance a complex tightly regulated process. The sterol-transporter ATP Binding Cassette transporter G1 (ABCG1) critical for maintaining normal cellular lipid homeostasis. After liver intestine, lung 3rd most active lipid-secreting organ. Pulmonary alveolar proteinosis (PAP) rare characterized by accumulation surfactant lipids proteins alveoli resulting respiratory distress. For almost all reported cases underlying unknown. Synthesis secretion restricted epithelial type 2 pneumocytes (T2 cells). However, subsequent clearance from hypophase dependent upon both T2 cells macrophages. Both macrophages express high levels ABCG1. Abcg1-/- mice severe lipidosis, elevated multiple species presence lipid-loaded containing abnormal lamellar bodies. Recent advances have also implicated role innate immune system pathogenesis PAP. We recently demonstrated that lungs exhibit profound response associated local increase natural antibody-secreting B-1 B circulating antibodies. Here we show lacking ABCG1 specifically bodies altered phospholipid homeostasis, gene expression. Collectively, our results suggest metabolism expansion antibodies may provide novel model which study PAP, risk developing disease.