Cystic Fibrosis Heart

Title: The role of CFTR in cardiomyocytes Three Specific Aims Aim 1. Identify the mechanisms of hERG modulation resulting from CFTR mutations and the effect of CFTR correctors. Aim 2. Determine the mechanisms by which CFTR modulates cardiomyocyte mitochondrial signaling in MHC-CFTR-/- mice, and F508d mutant mice and iPS-CM models. Aim 3. Examine the susceptibility to QT prolongation and VT in CF pigs. EKG will be recorded for newborn CFTR-/- piglets stressed with epinephrine, followed by tissue analysis.