Table 1.

Rare variants in idiopathic pulmonary fibrosis.

Gene	Gene function	Pathological consequence of mutation	Reference
SFTPC	Component of surfactant fluid	Altered trafficking and disrupted proteostasis, increased endoplasmic reticulum (ER) stress	(25–27)
SFTPA2	To modulate innate and adaptive immunity	Increase in ER stress	(15, 25, 28)
ABCA3	Transport of lipids across plasma membrane	Retention of lipids in the ER, ER stress, and apoptotic signaling	(29–31)
TERT	Enzyme in telomerase complex	Telomere shortening	(7, 18, 27, 32–36)
TERC	Template in telomerase complex	Telomere shortening	(7, 18, 27, 32–37)
DKC1	Stabilization of the template in telomerase complex	Telomere shortening	(19, 27, 38)
TINF2	Telomere maintenance	Telomere shortening	(20, 39)
RTELI	DNA helicase	Telomere shortening	(21, 22, 40)
PARN	mRNA stability	Telomere shortening	(21, 24)