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Science 1 April 1994: Vol. 264. no. 5155, pp. 92 - 95 DOI: 10.1126/science.8140421
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Articles
Science, Vol 264, Issue 5155, 92-95
Copyright © 1994 by American Association for the Advancement of Science
Requirement of human renal water channel aquaporin-2 for vasopressin-dependent concentration of urine
PM Deen,
MA Verdijk,
NV Knoers,
B Wieringa,
LA Monnens,
CH van Os,
and
BA van Oost
Department of Cell Physiology, University of Nijmegen, Netherlands.
Concentration of urine in mammals is regulated by the antidiuretic hormone vasopressin. Binding of vasopressin to its V2 receptor leads to the insertion of water channels in apical membranes of principal cells in collecting ducts. In nephrogenic diabetes insipidus (NDI), the kidney fails to concentrate urine in response to vasopressin. A male patient with an autosomal recessive form of NDI was found to be a compound heterozygote for two mutations in the gene encoding aquaporin-2, a water channel. Functional expression studies in Xenopus oocytes revealed that each mutation resulted in nonfunctional water channel proteins. Thus, aquaporin-2 is essential for vasopressin-dependent concentration of urine.
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- Disruption of lens fiber cell architecture in mice expressing a chimeric AQP0-LTR protein.
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FASEB J
14, 2207-2212
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- Molecular characterization of a novel UT-A urea transporter isoform (UT-A5) in testis.
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Am J Physiol Cell Physiol
279, C1425-C1431
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- Importance of aquaporin-2 expression levels in genotype -phenotype studies in nephrogenic diabetes insipidus.
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Am J Physiol Renal Physiol
279, F778-F784
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- Functional impairment of lens aquaporin in two families with dominantly inherited cataracts.
- P. Francis, J.-J. Chung, M. Yasui, V. Berry, A. Moore, M. K. Wyatt, G. Wistow, S. S. Bhattacharya, and P. Agre (2000)
Hum. Mol. Genet.
9, 2329-2334
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- Aldose Reductase-Deficient Mice Develop Nephrogenic Diabetes Insipidus.
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Mol. Cell. Biol.
20, 5840-5846
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- Dissociation between urine osmolality and urinary excretion of aquaporin-2 in healthy volunteers.
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Nephrol. Dial. Transplant.
15, 1155-1161
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- Role of aquaporins in alveolar fluid clearance in neonatal and adult lung, and in oedema formation following acute lung injury: studies in transgenic aquaporin null mice.
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J. Physiol.
525, 771-779
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- Key Roles of Renal Aquaporins in Water Balance and Water-Balance Disorders.
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Physiology
15, 136-143
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- Skeletal muscle function and water permeability in aquaporin-4 deficient mice.
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Am J Physiol Cell Physiol
278, C1108-C1115
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- Functional Characterization of the Molecular Defects Causing Nephrogenic Diabetes Insipidus in Eight Families.
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J. Clin. Endocrinol. Metab.
85, 1703-1710
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- Aquaporin Water Channels in Kidney.
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11, 764-777
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- Aquaporin 2 is a vasopressin-independent, constitutive apical membrane protein in rat vas deferens.
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Am J Physiol Cell Physiol
278, C791-C802
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- Hypertonic Induction of Aquaporin-5 Expression through an ERK-dependent Pathway.
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275, 9070-9077
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- Decreased vasopressin-mediated renal water reabsorption in rats with chronic aldosterone-receptor blockade.
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Am J Physiol Renal Physiol
278, F246-F256
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- Respiratory Aquaporins in Lung Inflammation . The Night Is Young.
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22, 8-10
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- Structure and function of aquaporin water channels.
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Am J Physiol Renal Physiol
278, F13-F28
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274, 34825-34831
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J. Pharmacol. Exp. Ther.
291, 920-923
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Am J Physiol Renal Physiol
277, F734-F741
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- At Physiological Expression Levels the Kidd Blood Group/Urea Transporter Protein Is Not a Water Channel.
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274, 30228-30235
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- Clinical Presentation and Follow-Up of 30 Patients with Congenital Nephrogenic Diabetes Insipidus.
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J. Am. Soc. Nephrol.
10, 1958-1964
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- Decreased Abundance of Collecting Duct Aquaporins in Post-Ischemic Renal Failure in Rats.
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J. Am. Soc. Nephrol.
10, 1658-1668
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