Table 2:

Top canonical pathways and upstream regulators from pathway analysis

Top Canonical Pathways	p-value	overlap
Pre-Luma/Iva vs Control
Role of macrophages, fibroblasts and endothelial cells	9.30E-06	5.6%
IL-10 signaling	1.24E-04	10.1%
Th1 and Th2 activation pathway	1.74E-04	6.1%
Hepatic fibrosis/hepatic stellate cell activation	1.82E-04	6.1%
II-6 signaling	2.39E-04	7.0%
Post-Luma/Iva vs Control
Role of macrophages, fibroblasts and endothelial cells	1.75E-04	3.0%
Role of pattern recognition receptors	2.29E-04	4.6%
Role JAK family kinases in IL-6 type cytokine signaling	5.73E-04	12.0%
Role of osteoblasts, osteoclasts and chrondrocytes	7.83E-04	3.1%
Paxillin signaling	7.99E-04	4.5%
Pre vs Post-Luma/lva
RhoA Signaling	4.30E-02	0.8%
Integrin Signaling	7.37E-02	0.5%
Actin cytoskeleton signaling	7.68E-02	0.5%
Protein Kinase A signaling	1.29E-01	0.3%
Axonal guidance signaling	1.49E-01	0.2%
Responder vs non-responders
EIF2 Signaling	4.46E-06	9.5 %
Oxidative Phosphorylation	4.98E-04	10.1 %
Mitochondrial Dysfunction	7.80E-04	8.2 %
IL-17A Signaling in Gastric Cells	8.30E-04	20.0 %
Regulation of eIF4 and p70S6K Signaling	1.07E-03	8.3 %
Top Upstream Regulators	p-value	predicted activation
Pre-Luma/Iva vs Control
Lipopolysaccharide	2.96E-14	activated
TNK	1.78E-11	activated
Immunoglobulin	3.67E-11	inhibited
TGM2	5.02E-09	activated
IL-1β	6.2E-08	activated
Post-Luma/Iva vs Control
Lipopolysaccharide	9.88E-11	activated
OSM	1.61E-09	activated
Cg	1.59E-08
PGR	5.50E-07
IL-1β	8.04E-07	activated
Pre vs Post-Luma/Iva
I-BET-151	1.59E-03
HAND2	1.62E-02
TBX5	1.89E-02
MEF2C	2.21 E-02
MYOCD	2.60E-02
Responder vs non-responders
tomelukast	3.05E-05
RICTOR	3.23E-05	Inhibited
HNF4A	4.57E-05
Ifnar	9.36E-05
STAT2	1.71E-04

Luma/Iva – lumacaftor/ivacaftor