Periostin-mediated dysfunction of myocardial structural and functional responses appeared to be dependent on NAP1L2, which induced inhibition of SIRT3 that deacetylates H3K27ac on the promoters of the BCAA catabolism-related enzymes BCAT2 and PP2CM, resulting in the accumulation of BCAAs in CF. Here, PPM1K is linked to cystic fibrosis.