“…The nature of some mutations associated with IPF, such as those in the genes encoding surfactant proteins (SFTPs) A2 (Wang et al, 2009) and SFTPC (Lawson et al, 2004; Nogee et al, 2001; Nureki et al, 2018; Thomas et al, 2002), suggests that injury to type II alveolar epithelial (ATII) cells, the surfactant-producing cells of the alveoli, is critical to pathogenesis (Fingerlin et al, 2013; Seibold et al, 2011; Yang et al, 2015; Zhang et al, 2011). Eight percent to 15% of patients with familial IPF have heterozygous mutations in the reverse transcriptase (hTERT) or RNA component (hTERC) of telomerase (Alder et al, 2008, 2011, 2015a; Armanios, 2012a, 2012b, 2007). Furthermore, several susceptibility loci have been identified through exome sequencing that affect telomere length (Stuart et al, 2015).…”