Urinary concentrating ability in patients with Jk(a-b-) blood type who lack carrier-mediated urea transport.
Water homeostasis is regulated in large part by the proper operation of the urinary concentrating mechanism. In the renal inner medulla, urea recycling from the inner medullary collecting duct to the inner medullary interstitium is thought to be essential for the production of a concentrated urine; however, it has not been possible to test this hypothesis in humans. Recently, a unique combination of genetic abnormalities has been described: absence of Kidd blood group antigens and absence of carrier-mediated urea transport in erythrocytes. Because animal studies indicate a similarity between urea transport in red blood cells and the nephron, it was postulated that patients without the Kidd antigen might lack facilitated urea transport in their kidneys. Hence, their ability to concentrate urine maximally was measured. Current models of nephron function would predict that in the complete absence of urea transport, the maximal concentrating ability would be around 800 to 900 mosM/kg H2O. Two homozygous patients had a moderate decrease in maximal concentrating ability (UosM,max = 819 mosM/kg H2O); a heterozygote also had some limitation. These studies raise the possibility that the erythrocyte urea transporter and the kidney urea transporter are encoded by a single gene (detected by the mutational loss of the Kidd antigen) and that a lack of facilitated urea transport impairs urea recycling in the kidney and, hence, maximal urinary concentrating ability.
Sands
J M
JM
Department of Medicine, Emory University School of Medicine, Atlanta, GA 30322.
Gargus
J J
JJ
Fröhlich
O
O
Gunn
R B
RB
Kokko
J P
JP
D000293:Adolescent; D001693:Biological Transport, Active / Q000235:genetics; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007667:Kidd Blood-Group System / Q000235:genetics*; D007671:Kidney Concentrating Ability / Q000235:genetics / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D014508:Urea / Q000378:metabolism*; D014882:Water-Electrolyte Balance / Q000235:genetics / Q000502:physiology
D007667:Kidd Blood-Group System; D014508:Urea