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CAN WE LIVE WITHOUT A FUNCTIONAL RENIN‐ANGIOTENSIN SYSTEM?
Author(s) -
Pierre Corvol,
Annie Michaud,
Olivier Gribouval,
JeanMarie Gasc,
MarieClaire Gubler
Publication year - 2008
Publication title -
clinical and experimental pharmacology and physiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.752
H-Index - 103
eISSN - 1440-1681
pISSN - 0305-1870
DOI - 10.1111/j.1440-1681.2008.04891.x
Subject(s) - renin–angiotensin system , anuria , endocrinology , medicine , kidney , biology , gestation , hyperplasia , phenotype , gene , pregnancy , blood pressure , genetics
SUMMARY1 In mice, inactivation of any of the components of the renin‐angiotensin system (i.e. renin, angiotensin‐converting enzyme, angiotensinogen and AT 1 receptor) is dispensable for survival at birth. Animals can survive although they are more sensitive to salt depletion than the wild type mice. 2 Renal tubular dysgenesis (RTD) is a human disease consisting of severe abnormalities of renal tubular development and resulting in profound anuria and perinatal death. 3 Familial RTD is an autosomal recessive disease due to genetic defects in any of the constituents of the renin system. 4 Complete gene inactivation of the renin system in RTD leads to neonatal anuria and death. Proximal tubules are almost absent; renal artery hyperplasia is found in all cases of RTD. An intense stimulation of renin gene expression is noted in the kidney of patients with mutations affecting angiotensinogen, angiotensin‐converting enzyme and AT 1 receptor. 5 The more severe phenotype in humans than in mice devoid of a functional renin system may be attributable to the difference in nephrogenesis between mice and humans. In mice, nephrogenesis is completed 2 weeks after birth, whereas in humans it is completed before birth, at 38 weeks of gestation.