GBA2-encoded β-glucosidase activity is involved in the inflammatory response to Pseudomonas aeruginosa.

ABSTRACT: Current anti-inflammatory strategies for the treatment of pulmonary disease in cystic fibrosis (CF) are limited; thus, there is continued interest in identifying additional molecular targets for therapeutic intervention. Given the emerging role of sphingolipids (SLs) in various respiratory disorders, including CF, drugs that selectively target the enzymes associated with SL metabolism are under development. Miglustat, a well-characterized iminosugar-based inhibitor of β-glucosidase 2 (GBA2), has shown promise in CF treatment because it reduces the inflammatory response to infection by P. aeruginosa and restores F508del-CFTR chloride channel activity. This study aimed to probe the molecular basis for the anti-inflammatory activity of miglustat by examining specifically the role of GBA2 foll

SUBMITTER: Loberto N 

PROVIDER: S-EPMC4139313 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature