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Article

Potentiation of ABCA3 lipid transport function by ivacaftor and genistein

18 Jun 2019

Abstract excerpt

ABCA3 is a phospholipid transporter implicated in pulmonary surfactant homoeostasis and localized at the limiting membrane of lamellar bodies, the storage compartment for surfactant in alveolar type II cells. Mutations in ABCA3 display a common genetic cause for diseases caused by surfactant deficiency like respiratory distress in neonates and interstitial lung disease in children and adults, for which currently...

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PMID: 31210424
DOI: 10.1111/jcmm.14397

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