Table 1

ET-1+/+

kidney: renal cysts, renal interstitial fibrosis, glomerulosclerosis

lung: fibrosis and chronic inflammation

[2, 3, 12]

endothelium specific ET+/+

vascular remodelling and endothelial dysfunction

[13]

cardiac specific ET+/+

inflammatory cardiomyopathy

[14]

ET-2+/+

glomerulosclerosis

[15]

ET-3+/+

hyperpigmentation

[17]

ET-1+/+eNOS-/-

elevated blood pressure

[11]

Knockout Models

Phenotype

References

ET-1-/

craniopharyngeal and cardiovascular malformations

[18–20]

ET-1+/-

elevated blood pressure

[18, 23–25]

collecting duct-specific ET-1-/-

elevated blood pressure

[27, 28]

cardiomyocyte-specific ET-1-/-

dilatative cardiomyopathy

[30, 31]

ET-3-/-

aganglionic megacolon, pigmentary disorders

[16, 32]

ETAR-/-

craniopharyngeal and cardiovascular malformations

[21]

ETBR-/-

aganglionic megacolon, pigmentary disorders

[16, 34]

rescued ETBR-/-

salt-sensitive hypertension

[35, 36, 38]

diabetic rescued ETBR-/-

low-renin hypertension, progressive renal failure

[39]

endothelial cell-specific ETBR-/-

endothelial dysfunction, normotensive on high-salt diet

[40]

collecting duct-specific ETBR-/

elevated blood pressure, increasing blood pressure and impaired sodium

excretion on high-salt diet

[41]

ECE-1-/-

severe cardiac developmental disorders, craniofacial abnormalities, aganglionic megacolon, pigmentary disorders

[45]

ECE-2-/-

healthy phenotype

[46]

ECE-1-/-;ECE-2-/-

worsened ECE-1-/-embryonic phenotype

[46]