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Table 1

From: Pathophysiology of the endothelin system - lessons from genetically manipulated animal models

Transgenic Models Phenotype References
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 [1820]
ET-1+/- elevated blood pressure [18, 2325]
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
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]