Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis.

ABSTRACT: Rationale: In cystic fibrosis the major cause of morbidity and mortality is lung disease characterized by inflammation and infection. The influence of sphingolipid metabolism is poorly understood with a lack of studies using human airway model systems.Objectives: To investigate sphingolipid metabolism in cystic fibrosis and the effects of treatment with recombinant human acid ceramidase on inflammation and infection.Methods: Sphingolipids were measured using mass spectrometry in fully differentiated cultures of primary human airway epithelial cells and cocultures with Pseudomonas aeruginosa. In situ activity assays, Western blotting, and quantitative PCR were used to investigate function and expression of ceramidase and sphingomyelinase. Effects of treatm

SUBMITTER: Gardner AI 

PROVIDER: S-EPMC7560813 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature