Acid Sphingomyelinase Inhibitors Normalize Pulmonary Ceramide and Inflammation in Cystic Fibrosis

Therapeutic Efficacy and Safety of Amitriptyline in Patients with Cystic Fibrosis

(2009) Joachim Riethmüller et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

Defective CFTR increases synthesis and mass of sphingolipids that modulate membrane composition and lipid signaling

(2009) Hiroko Hamai et al.   JOURNAL OF LIPID RESEARCH

cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-Pick type C cells

(2008) Mary E. Manson et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Cystic Fibrosis Fatty Acid Imbalance Is Linked to Ceramide Deficiency and Corrected by Fenretinide

(2008) Claudine Guilbault et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Lysosomal Targeting and Trafficking of Acid Sphingomyelinase to Lipid Raft Platforms in Coronary Endothelial Cells

(2008) Si Jin et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease

(2008) Gerd Döring et al.   CELLULAR MICROBIOLOGY

Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis

(2008) Volker Teichgräber et al.   NATURE MEDICINE

Fenretinide Corrects Newly Found Ceramide Deficiency in Cystic Fibrosis

(2007) Claudine Guilbault et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Identification of New Functional Inhibitors of Acid Sphingomyelinase Using a Structure−Property−Activity Relation Model

(2007) Johannes Kornhuber et al.   JOURNAL OF MEDICINAL CHEMISTRY