It is hypothesized that ACO1 (cytoplasm-localized aconitase isoform) plays a critical role in the IPF metabolome, more specifically, in the proliferation of certain cells and in the pathogenesis of IPF given that enzymatic functions in mitochondria are impaired in IPF (Zhao et al., 2017). Here, ACO1 is linked to idiopathic pulmonary fibrosis.