Moreover, a key enzyme for FAO, peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α), was upregulated in IPF macrophages; (3) chimeric mice, WT with Mcu+/−-bone marrow (BM), showed resistance to BLM-induced lung fibrosis. This evidence concerns the gene PPARGC1A and idiopathic pulmonary fibrosis.