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Citations to this article

Altered activity of the nucleotide regulatory site in the parathyroid hormone-sensitive adenylate cyclase from the renal cortex of a patient with pseudohypoparathyroidism.
M K Drezner, W M Burch Jr
M K Drezner, W M Burch Jr
Published December 1, 1978
Citation Information: J Clin Invest. 1978;62(6):1222-1227. https://doi.org/10.1172/JCI109242.
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Research Article

Altered activity of the nucleotide regulatory site in the parathyroid hormone-sensitive adenylate cyclase from the renal cortex of a patient with pseudohypoparathyroidism.

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Abstract

A series of clinical studies suggest that the primary defect underlying pseudohypoparathyroidism is an abnormality of the parathyroid hormone-receptor-adenylate cyclase complex of the renal cortical cell plasma membrane. In the present study we compared parathyroid hormone-stimulated adenylate cyclase activity in membrane preparations from the renal cortex of three controls and a patient with pseudohypoparathyroidism. In the pseudohypoparathyroid preparation the Km for ATP was significantly greater and parathyroid hormone elicited markedly diminished adenylate cyclase activity at a subsaturating concentration of ATP. In contrast, the dose-response effect of enzyme activity to parathyroid hormone was the same in the control preparations, and that of the pseudohypoparathyroidism kidney, at a saturating concentration of ATP. The apparent alteration in enzyme kinetics, however, was normalized upon addition of guanosine 5'-triphosphate to the reaction mixtures. These results indicate that the defect in the parathyroid hormone-receptor-adenylate cyclase complex of the renal cell membranes, in our patient with pseudohypoparathyroidism, is an abnormal nucleotide receptor site of decreased activity. Such a defect may result in partial uncoupling of the parathyroid hormone receptor and adenylate cyclase, rendering the organ refractory to hormonal stimulation.

Authors

M K Drezner, W M Burch Jr

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Total citations by year

Year: 2010 2008 2006 2000 1999 1998 1997 1990 1989 1988 1987 1986 1985 1984 1983 1982 1981 1980 1979 1978 Total
Citations: 1 2 1 1 1 1 1 1 3 2 3 3 3 3 5 3 4 5 2 1 46
Citation information
This citation data is accumulated from CrossRef, which receives citation information from participating publishers, including this journal. Not all publishers participate in CrossRef, so this information is not comprehensive. Additionally, data may not reflect the most current citations to this article, and the data may differ from citation information available from other sources (for example, Google Scholar, Web of Science, and Scopus).

Citations to this article (46)

Title and authors Publication Year
Vitamins & Hormones
KJ Hare, FK Knop
Vitamins & Hormones 2010
Hypoparathyroidism
D Shoback
New England Journal of Medicine 2008
Principles of Bone Biology
CJ Rosen, T Niu
Principles of Bone Biology 2008
Multihormonal Resistance to Parathyroid Hormone, Thyroid Stimulating Hormone, and Other Hormonal and Neurosensory Stimuli in Patients with Pseudohypoparathyroidism
JL Wimeau, AS Balavoine, M Ladsous, FL Velayoudom-Cephise, V Vlaeminck-Guillem
Journal of Pediatric Endocrinology and Metabolism 2006
Proximal Tubular Phosphate Reabsorption: Molecular Mechanisms
H Murer, N Hernando, I Forster, J Biber
Physiological reviews 2000
Hormone Resistance Syndromes
JL Jameson
1999
Advances in Protein Chemistry
CM Silva, J Isgaard, MO Thorner
Advances in Protein Chemistry 1998
Targeted Disruption of Gnas in Embryonic Stem Cells*
WF Schwindinger, KJ Reese, AM Lawler, JD Gearhart, MA Levine
Endocrinology 1997
Pseudohypoparathyreoidismus und Nebennierenrindeninsuffizienz: Ein Fall von Multipler Endokrinopathie bei peripherer Hormonresistenz
P Ridderskamp, R Schlaghecke
Klinische Wochenschrift 1990
Decreased bioactivity of the guanine nucleotide-binding protein that stimulates adenylate cyclase in hearts from cardiomyopathic Syrian hamsters
PD Kessler, AE Cates, CV Dop, AM Feldman
Journal of Clinical Investigation 1989
Altered response of adenylate cyclase to parathyroid hormone during compensatory renal growth
CL Milanes, N Pernalete, R Starosta, M Perez-Gonzalez, V Paz-Martinez, E Bellorin-Font
Kidney International 1989
Hypocalcemic and hypercalcemic disorders in children
G Kainer, JC Chan
Current Problems in Pediatrics 1989
Vitamin D
A Portale, Β Halloran, E Lonergan, R Morris
Vitamin D 1988
Oligosymptomatic hypocalcaemia in a patient with impaired parathyroid hormone activity
A Lenox-Smith, W Smith, GA Ferns, C Moniz
Journal of the Royal Society of Medicine 1988
Phosphate homeostasis
PJ Butterworth
Molecular Aspects of Medicine 1987
Clinical Disorders of Membrane Transport Processes
TE Andreoli, JF Hoffman, DD Fanestil, SG Schultz
1987
Hormone Resistance and Other Endocrine Paradoxes
MP Cohen, PP Foà
1987
Proceedings of the 1985 Laurentian Hormone Conference
RM Carey, S Sen
Proceedings of the 1985 Laurentian Hormone Conference 1986
The Receptors
RG Rosenfeld, RL Hintz
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Clinical Endocrinology
A Labhart
Clinical Endocrinology 1986
Isoproterenol-sensitive adenylate cyclase in glomeruli isolated from young and adult rat renal cortex
C Judes, JJ Helwig, C Bollack, P Mandel
General Pharmacology: The Vascular System 1985
Contemporary Endocrinology
SH Ingbar
1985
Homeostasis, Nephrotoxicity, and Renal Anomalies in the Newborn
J Strauss
1985
Impaired formation of beta-adrenergic receptor-nucleotide regulatory protein complexes in pseudohypoparathyroidism
JA Heinsimer, AO Davies, RW Downs, MA Levine, AM Spiegel, MK Drezner, AD Lean, KA Wreggett, MG Caron, RJ Lefkowitz
Journal of Clinical Investigation 1984
Phosphate and Mineral Metabolism
SG Massry, G Maschio, E Ritz
1984
Renal refractoriness to PTH in experimental Fanconi syndrome: Evidence for intact adenylate cyclase activation
H Wald, MM Popovtzer
Pflügers Archiv - European Journal of Physiology 1984
Deficient adenylate cyclase regulatory protein in renal membranes from a patient with pseudohypoparathyroidism
RW Downs, MA Levine, MK Drezner, WM Burch, AM Spiegel
Journal of Clinical Investigation 1983
PSEUDOHYPOPARATHYROIDISM: INHERITANCE AND EXPRESSION OF DEFICIENT RECEPTOR-CYCLASE COUPLING PROTEIN ACTIVITY
JA Fischer, HR Bourne, MA Dambacher, F Tschopp, R Meyer, JP Devogelaer, EA Werder, CN Deuxchaisnes
Clinical Endocrinology 1983
Introduction: The clinical significance of peptide hormone receptors
KJ Gatt, ML Dufau
Clinics in Endocrinology and Metabolism 1983
Dünndarm A
F Bazzoli, R Böhmer, WF Caspary, H Dombrowski, B Eckhardt, B Elsenhans, A Encke, A Ferguson, W Forth, H Fromm, A Gangl, S Gutschmidt, U Hopfer, GJ Kreijs, B Lembcke, KH zum Büschenfelde, G Mödder, G Nell, HF Otto, H Peerenboom, G Rehner, G Rettenmaier, EO Riecken, JW Robinson, K Rommel, H Ruppin, J Seifert, KH Soergel, S Strobel, L Vollrath, R Wanitschke, HJ Weis
1983
Teratogenesis and Reproductive Toxicology
EM Johnson, DM Kochhar
1983
Albright's hereditary osteodystrophy: A review
N Fitch, JM Opitz, J Herrmann
American Journal of Medical Genetics 1982
Disorders of Mineral Metabolism
RK Rude, FR Singer
Disorders of Mineral Metabolism 1982
Regulation of Phosphate and Mineral Metabolism
SG Massry, JM Letteri, E Ritz
1982
BASAL GANGLIA CALCIFICATION IN PSEUDOHYPOPARATHYROIDISM TYPE II
R Windeck, U Menken, G Benker, D Reinwein
Clinical Endocrinology 1981
Down syndrome fibroblasts are hyperresponsive to beta-adrenergic stimulation
JD McSwigan, DR Hanson, A Lubiniecki, LL Heston, JR Sheppard
Proceedings of the National Academy of Sciences 1981
Cell cycle changes in the adenylate cyclase of C6 glioma cells
RF Howard, JR Sheppard
The Journal of Cell Biology 1981
Contemporary Nephrology
S Klahr, SG Massry
1981
Adenylate cyclase responsiveness to hormones in various portions of the human nephron
D Chabardès, M Gagnan-Brunette, M Imbert-Teboul, O Gontcharevskaia, M Montégut, A Clique, F Morel
Journal of Clinical Investigation 1980
Cellular Action of Vasopressin in Medullary Tubules of Mice with Hereditary Nephrogenic Diabetes Insipidus
BA Jackson, RM Edwards, H Valtin, TP Dousa
Journal of Clinical Investigation 1980
Demonstration of a lack of change in serum 1 alpha,25-dihydroxyvitamin D in response to parathyroid extract in pseudohypoparathyroidism
PW Lambert, BW Hollis, NH Bell, S Epstein
Journal of Clinical Investigation 1980
Studies on the attainment of normocalcemia in patients with pseudohypoparathyroidism
NA Breslau, DD Notman, JM Canterbury, AM Moses
The American Journal of Medicine 1980
Defect of Receptor–Cyclase Coupling Protein in Pseudohypoparathyroidism
Z Farfel, AS Brickman, HR Kaslow, VM Brothers, HR Bourne
New England Journal of Medicine 1980
The syndromes of primary hormone resistance
GF Verhoeven, JD Wilson
Metabolism 1979
Contemporary Metabolism
N Freinkel
1979
Physiology of Membrane Disorders
TE Andreoli, JF Hoffman, DD Fanestil
1978

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