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Previous Volume 330:178-181 January 20, 1994 Number 3 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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In 1963 Liddle et al.¹ described a disorder that simulated primary aldosteronism, characterized by severe hypertension and hypokalemia but with negligible secretion of aldosterone. They theorized that this was "a disorder in which the renal tubules transport ions with such abnormal facility that the end result simulates that of a mineralocorticoid excess." We describe a woman with this syndrome (the index case¹) in whom renal failure eventually developed and who received a cadaveric renal transplant at our institution in 1989. Her disorder resolved after transplantation, with normalization of the aldosterone and renin responses to salt restriction. The woman's extended . . . [Full Text of this Article]

Case Report

Methods

Results

Discussion

Source Information

From the Departments of Medicine (M.B.-V., J.J.C., D.G.W.) and Physiology (D.G.W.), the General Clinical Research Center (J.J.C.), and the Nephrology Research and Training Center (M.B.-V., J.J.C., D.G.W.), University of Alabama at Birmingham, and the Veterans Affairs Medical Center (D.G.W.), both in Birmingham, Ala. Presented in preliminary form at the 25th Annual Meeting of the American Society of Nephrology, Baltimore, November 15-18, 1992 (J Am Soc Nephrol 1992;3:517a. abstract).

Address reprint requests to Dr. Warnock at the Division of Nephrology, University of Alabama at Birmingham, UAB Station, Birmingham, AL 35294-0007.

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• Reinstein, E., Ciechanover, A. (2006). Narrative review: protein degradation and human diseases: the ubiquitin connection.. ANN INTERN MED 145: 676-684 [Abstract] [Full Text]  
• Ponda, M. P., Hostetter, T. H. (2006). Aldosterone Antagonism in Chronic Kidney Disease. CJASN 1: 668-677 [Full Text]  
• Staub, O., Verrey, F. (2005). Impact of Nedd4 Proteins and Serum and Glucocorticoid-Induced Kinases on Epithelial Na+ Transport in the Distal Nephron. J. Am. Soc. Nephrol. 16: 3167-3174 [Abstract] [Full Text]  
• Furuhashi, M., Kitamura, K., Adachi, M., Miyoshi, T., Wakida, N., Ura, N., Shikano, Y., Shinshi, Y., Sakamoto, K.-i., Hayashi, M., Satoh, N., Nishitani, T., Tomita, K., Shimamoto, K. (2005). Liddle's Syndrome Caused by a Novel Mutation in the Proline-Rich PY Motif of the Epithelial Sodium Channel {beta}-Subunit. J. Clin. Endocrinol. Metab. 90: 340-344 [Abstract] [Full Text]  
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• Otulakowski, G., Rafii, B., O'Brodovich, H. (2004). Differential Translational Efficiency of ENaC Subunits During Lung Development. Am. J. Respir. Cell Mol. Bio. 30: 862-870 [Abstract] [Full Text]  
• Luft, F. C. (2003). Mendelian Forms of Human Hypertension and Mechanisms of Disease. Clin Med Res 1: 291-300 [Abstract] [Full Text]  
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• Pratt, J. H., Ambrosius, W. T., Agarwal, R., Eckert, G. J., Newman, S. (2002). Racial Difference in the Activity of the Amiloride-Sensitive Epithelial Sodium Channel. Hypertension 40: 903-908 [Abstract] [Full Text]  
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• Snyder, P. M. (2002). The Epithelial Na+ Channel: Cell Surface Insertion and Retrieval in Na+ Homeostasis and Hypertension. Endocr. Rev. 23: 258-275 [Abstract] [Full Text]  
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• Luft, F. C. (2001). Molecular Genetics of Salt-Sensitivity and Hypertension. Drug Metab. Dispos. 29: 500-504 [Abstract] [Full Text]  
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• Scheinman, S. J., Guay-Woodford, L. M., Thakker, R. V., Warnock, D. G. (1999). Genetic Disorders of Renal Electrolyte Transport. NEJM 340: 1177-1187 [Full Text]  
• Persu, A., Barbry, P., Bassilana, F., Houot, A.-M., Mengual, R., Lazdunski, M., Corvol, P., Jeunemaitre, X. (1998). Genetic Analysis of the ß Subunit of the Epithelial Na+ Channel in Essential Hypertension. Hypertension 32: 129-137 [Abstract] [Full Text]  
• Dinudom, A., Harvey, K. F., Komwatana, P., Young, J. A., Kumar, S., Cook, D. I. (1998). Nedd4 mediates control of an epithelial Na+ channel in salivary duct cells by cytosolic Na+. Proc. Natl. Acad. Sci. USA 95: 7169-7173 [Abstract] [Full Text]  
• Melander, O., Orho, M., Fagerudd, J., Bengtsson, K., Groop, P.-H., Mattiasson, I., Groop, L., Hulthen, U. L. (1998). Mutations and Variants of the Epithelial Sodium Channel Gene in Liddle's Syndrome and Primary Hypertension. Hypertension 31: 1118-1124 [Abstract] [Full Text]  
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• Ismailov, I. I., Awayda, M. S., Berdiev, B. K., Bubien, J. K., Lucas, J. E., Fuller, C. M., Benos, D. J. (1996). Triple-barrel Organization of ENaC, a Cloned Epithelial Na[IMAGE] Channel. J. Biol. Chem. 271: 807-816 [Abstract] [Full Text]  
• Nakatsu, F., Sakuma, M., Matsuo, Y., Arase, H., Yamasaki, S., Nakamura, N., Saito, T., Ohno, H. (2000). A Di-leucine Signal in the Ubiquitin Moiety. POSSIBLE INVOLVEMENT IN UBIQUITINATION-MEDIATED ENDOCYTOSIS. J. Biol. Chem. 275: 26213-26219 [Abstract] [Full Text]