TABLE 1

Targeting developmental pathways in idiopathic pulmonary fibrosis

PathwayDrug/moleculeTargetEffect[Reference]
Wnt-β-cateninAnti-WISP1 antibodyWISP1Decreases fibrosis in bleomycin-induced fibrosis in mice[76]
siRNA against β-cateninβ-catenin[77]
ICG-001CBP[78]
Sonic HedgehogGDC-0449 (Vismodegib)Smoothened[79]
CyclopamineSmoothened[80]
GANT-61GLI[79]
PirfenidoneGLI-2Slow lung function decline, improves survival[6, 81]
FOXO3UCN-01Protein kinase CImproves lung function, survival and decreases fibrosis in bleomycin-induced fibrosis in mice[39]
YAP/TAZVerteporfinTEAD/YAP interactionDecreases fibrosis in mouse model of kidney fibrosis[45, 82]
Fasudil/KD205ROCKDecreases fibrosis in bleomycin-induced fibrosis in mice[83]

siRNA: small interfering RNA; CBP: cyclic AMP response-element binding protein; YAP: Yes-associated protein.