MBTPS1 and cystic fibrosis: Moreover, the involvement of S1P/S1P lyase in cystic fibrosis pathology is supported by the evidence that in a murine cystic fibrosis model, treatment with a specific SGPL1 inhibitor is able to alter S1P metabolism and results in a reduction of the lung inflammatory response to Pseudomonas aeruginosa (Veltman et al., 2016).