Table 1:

Genes regulating endothelial cell signaling pathways and PAH

Gene (Pro + or anti − PH)	Expression Change in PH				PH Phenotypes
	Rat	Mu	Human		Rat		Mu
	LEC	LEC	WL	LEC	MCT	SuHx	Basal	MCT	Hx	SuHx
ABCA1 (−)				↓ [113]	Agonist ↓ [113]
AMPK (−)		↓ [157]		↓ [157]					EC KO ↑ [157]
									Agonist ↓ [157]
BMPR2 (−)		↓ [41, 42]		↓ [43]			LEC KO ↑ [42]	Het KO ↑ [40]	EC KO ↑ [41]
							Het Mut ↑ [45]
Apelin (−)		↓ [49, 52]		↓ [49]			Admin ↓ [49]		Hom KO ↑ [52]
BOLA3 (−)		↓ [105]		↓ [105]			LEC KO ↑ [105]		LEC OE ↓ [105]
Cav1 (−)		↓ [75]		↓ [71, 72]			Hom KO ↑ [74]		EC KO ↑ [72]
CCL2 (+)				↑ [159]
CD74 (+)				↑ [167]	Inhib ↓ [167]
CD39 (−)				↓ [51]					Hom KO ↑ [51]
CLIC4 (+)	↑ [53]			↑ [53]					Hom KO ↓ [53]
CTGF (+)		↑ [170]							EC KO ↓ [170]
CXCL12 (+)		↑ [154]							EC KO ↓ [154]
CypA (+)		↑ [172]		↑ [172]			EC OE ↑ [172]
ET-1(+)			↑ [140]				OE ↑ [139]	Inhib ↓ [138]	OE ↑ [139]	OE ↑ [139]
FGF2 (+)				↑ [153]	Inhib ↓ [153]
HIF2a (+)		↑ [67]		↑ [68]	Inhib ↓ [68]	Inhib ↓ [68]			LEC KO ↓ [67]
									EC KO ↓ [65, 66]
IDO (−)		↓ [160]							EC KO ↑ [160]
									LEC OE ↓ [160]
MRTFA (+)		↑ [114]							Hom KO ↓ [114]
mTOR (−)			↓ [77]						Admin ↓ [77]
MEF2 (−)				↓ [115]	Agonist ↓ [115]	Agonist ↓ [115]
Notch1 (+)		↑ [89]		↑ [88]		Inhib ↓ [88]			EC KO ↑ [89]
Nur77 (−)			↓ [78]	↓ [78]		Agonist ↓ [78]
P130Cas (+)	↑ [82]	↑ [82]		↑ [82]	Inhib ↓ [82]				Inhib ↓ [82]
PCD4 (+)				↑ [54]						Hom KO ↓ [54]
PFKFB3 (+)	↑ [102]	↑ [102]		↑ [102]		Inhib ↓ [102]			Het / EC KO ↓ [102]
PHD2 (−)		↓ [17, 64]		↓ [17]			EC & HC KO ↑ [17]
							EC KO ↑ [64, 65]
PKG (−)							Hom KO ↑ [146]
PPARg (−)			↓ [58]				EC KO ↑ [63]
TCTP (+)	↑ [163]			↑ [163]
VEGFR (−)										Inhib ↑ [80]

Multiple EC genes are expressed differentially in animal models of PH and human PH and have been shown to positively (+) or negatively (−) regulate PH. Abbreviations: ABCA, ATP binding cassette subfamily A member; Admin, administration; AMPK, AMP-activated protein kinase; BMPR, bone morphogenic protein receptor; BOLA, bolA family member; Cav, caveolin; CCL, CC ligand; CLIC, chloride intracellular channel; CM, conditioned media; CTGF, connective tissue growth factor; CXCL, C-X-C motif chemokine; CypA, cyclophilin A; EC, endothelial cell; Exp, expression; FGF, fibroblast growth factor; HC, hematopoietic cell; Het, heterozygous; HIF, hypoxia-inducible factor; Hom, homozygous; Hx, hypoxia; Inhib, inhibitor; IDO, indoleamine 2,3-dioxygenase; IPAH, idiopathic pulmonary arterial hypertension; KO, knockout; LEC, lung endothelial cells; MCT, monocrotaline; MEF, myocyte enhancer factor; MRTFA, myocardin-related transcription factor A; mTOR, mammalian target of rapamycin; Mu, mouse; Mut, mutation; OE, overexpression; PAH, pulmonary arterial hypertension; PFKFB, 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase; PH, pulmonary hypertension; PHD, prolyl hydroxylase; PKG, protein kinase G; PPAR, peroxisome proliferator-activated receptor; SuHx, sugen/hypoxia; TCTP, translationally controlled tumor protein; VEGFR, vascular endothelial growth factor receptor; WL, whole lung.