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J. Biol. Chem., Vol. 277, Issue 40, 37871-37880, October 4, 2002
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From the Departments of ¶ Molecular Genetics, Biochemistry,
and Microbiology, ROMK is an apical K+
channel expressed in the thick ascending limb of Henle (TALH) and
throughout the distal nephron of the kidney. Null mutations in the
ROMK gene cause type II Bartter's syndrome, in which
abnormalities of electrolyte, acid-base, and fluid-volume homeostasis
occur because of defective NaCl reabsorption in the TALH. To understand
better the pathogenesis of type II Bartter's syndrome, we developed a
mouse lacking ROMK and examined its phenotype. Young null mutants had
hydronephrosis, were severely dehydrated, and ~95% died before 3 weeks of age. ROMK-deficient mice that survived beyond weaning grew to
adulthood; however, they had metabolic acidosis, elevated blood
concentrations of Na+ and Cl Molecular and Cellular Physiology,
§ Internal Medicine, and Environmental Health, the
University of Cincinnati College of Medicine,
Cincinnati, Ohio 45267-0524
, reduced blood
pressure, polydipsia, polyuria, and poor urinary concentrating ability.
Whole kidney glomerular filtration rate was sharply reduced, apparently
as a result of hydronephrosis, and fractional excretion of electrolytes
was elevated. Micropuncture analysis revealed that the single nephron
glomerular filtration rate was relatively normal, absorption of NaCl in
the TALH was reduced but not eliminated, and tubuloglomerular feedback
was severely impaired. These data show that the loss of ROMK in the mouse causes perturbations of electrolyte, acid-base, and fluid-volume homeostasis, reduced absorption of NaCl in the TALH, and impaired tubuloglomerular feedback.
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