Journal of Clinical Endocrinology & Metabolism, Vol 62, 221-224, Copyright © 1986 by Endocrine Society

ARTICLES

Evidence for normal antidiuretic responses to endogenous and exogenous arginine vasopressin in patients with guanine nucleotide-binding stimulatory protein-deficient pseudohypoparathyroidism

AM Moses, RS Weinstock, MA Levine and NA Breslau

In six patients with pseudohypoparathyroidism (PHP) who were deficient in guanine nucleotide-binding stimulatory protein (Ns) activity, the response to endogenous arginine vasopressin (AVP) was tested during water deprivation. Hourly plasma osmolality (Posm), urinary osmolality (Uosm), and urinary AVP (UAVP) values were compared to those in normal subjects. The Uosm vs. Posm and the UAVP vs. Uosm relationships of the patients were all within the normal range. Four patients with Ns- deficient PHP were subjected to maintained water loads and infused with AVP at three different rates for 1 h each to assess their responses to exogenous AVP. Urinary volume and osmolality values from the final 30 min of each infusion rate were measured. All volume values except 1 were within 1.6 SD of normal, and all osmolality values except 1 were within 1.1 SD of normal. In conclusion, these studies indicate that these six patients with Ns-deficient PHP are not resistant to the antidiuretic (cAMP-mediated) action of endogenous or exogenous AVP, in contrast to the previously documented resistance of patients with Ns- deficient PHP to the actions of PTH, TSH, glucagon, and gonadotropins.

This article has been cited by other articles:

				A. Plagge, G. Kelsey, and E. L Germain-Lee Physiological functions of the imprinted Gnas locus and its protein variants G{alpha}s and XL{alpha}s in human and mouse J. Endocrinol., February 1, 2008; 196(2): 193 - 214. [Abstract] [Full Text] [PDF]

				R. A. Fenton and M. A. Knepper Mouse Models and the Urinary Concentrating Mechanism in the New Millennium Physiol Rev, October 1, 2007; 87(4): 1083 - 1112. [Abstract] [Full Text] [PDF]

				L. S. WEINSTEIN, M. CHEN, and J. LIU Gs{alpha} Mutations and Imprinting Defects in Human Disease Ann. N.Y. Acad. Sci., June 1, 2002; 968(1): 173 - 197. [Abstract] [Full Text] [PDF]

				L. S. Weinstein, S. Yu, D. R. Warner, and J. Liu Endocrine Manifestations of Stimulatory G Protein {alpha}-Subunit Mutations and the Role of Genomic Imprinting Endocr. Rev., October 1, 2001; 22(5): 675 - 705. [Abstract] [Full Text] [PDF]

				L. S. Weinstein, S. Yu, and C. A. Ecelbarger Variable imprinting of the heterotrimeric G protein Gs alpha -subunit within different segments of the nephron Am J Physiol Renal Physiol, April 1, 2000; 278(4): F507 - F514. [Abstract] [Full Text] [PDF]

				C. A. Ecelbarger, S. Yu, A. J. Lee, L. S. Weinstein, and M. A. Knepper Decreased renal Na-K-2Cl cotransporter abundance in mice with heterozygous disruption of the Gsalpha gene Am J Physiol Renal Physiol, August 1, 1999; 277(2): F235 - F244. [Abstract] [Full Text] [PDF]

				S. Yu, D. Yu, E. Lee, M. Eckhaus, R. Lee, Z. Corria, D. Accili, H. Westphal, and L. S. Weinstein Variable and tissue-specific hormone resistance in heterotrimeric Gs protein alpha -subunit (Gsalpha ) knockout mice is due to tissue-specific imprinting of the Gsalpha gene PNAS, July 21, 1998; 95(15): 8715 - 8720. [Abstract] [Full Text] [PDF]