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 | |
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 | |
ET-1+/- | elevated blood pressure | |
collecting duct-specific ET-1-/- | elevated blood pressure | |
cardiomyocyte-specific ET-1-/- | dilatative cardiomyopathy | |
ET-3-/- | aganglionic megacolon, pigmentary disorders | |
ETAR-/- | craniopharyngeal and cardiovascular malformations | [21] |
ETBR-/- | aganglionic megacolon, pigmentary disorders | |
rescued ETBR-/- | salt-sensitive hypertension | |
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] |