Deficient adenylate cyclase regulatory protein in renal membranes from a patient with pseudohypoparathyroidism.
Open Access
- 1 February 1983
- journal article
- research article
- Published by American Society for Clinical Investigation in JCI Insight
- Vol. 71 (2), 231-235
- https://doi.org/10.1172/jci110763
Abstract
Recent studies have established that some patients with pseudohypoparathyroidism have a deficiency of the adenylate cyclase regulatory protein (the G unit) in plasma membranes from erythrocytes, platelets, and fibroblasts. We have directly measured the activity of the G unit in renal membranes from a patient with pseudohypoparathyroidism who, in addition to parathyroid hormone resistance, has resistance to thyrotropin and gonadotropins. Erythrocyte membrane G unit activity was 57% that of control erythrocyte membranes. Lubrol PX extracts of renal membranes had only 30% of the G unit activity of control renal membrane extracts, whether assayed with sodium fluoride or guanosine-5'-O-(3-thiotriphosphate) (GTP-gamma-S). In cholate extracts, the G unit activity was 37 and 48% of control with fluoride or GTP-gamma-S, respectively. Cholera toxin-dependent incorporation of [32P]ADP-ribose into the 42,000-Mr subunit of the G unit was decreased in renal membranes from the patient compared with control renal membranes. The data demonstrate that the membrane G unit deficiency in pseudohypoparathyroidism extends to the cells of a clinically relevant parathyroid hormone target tissue.This publication has 24 references indexed in Scilit:
- Genetic evidence that cholera toxin substrates are regulatory components of adenylate cyclase.Journal of Biological Chemistry, 1978
- ADP-ribosylation of membrane proteins catalyzed by cholera toxin: basis of the activation of adenylate cyclase.Proceedings of the National Academy of Sciences, 1978
- PARTIAL GONADOTROPHIN-RESISTANCE IN PSEUDOHYPOPARATHYROIDISMActa Endocrinologica, 1978
- Selection of a Variant Lymphoma Cell Deficient in Adenylate CyclaseScience, 1975
- A highly sensitive adenylate cyclase assayAnalytical Biochemistry, 1974
- Thyroid Dysfunction in PseudohypoparathyroidismJournal of Clinical Endocrinology & Metabolism, 1971
- Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4Nature, 1970
- The Effect of Parathyroid Hormone on the Concentration of Adenosine 3',5'-Monophosphate in Skeletal Tissue in VitroJournal of Biological Chemistry, 1970
- Pseudohypoparathyroidism: defective excretion of 3′,5′-AMP in response to parathyroid hormoneJCI Insight, 1969
- Renal Adenyl Cyclase: Anatomically Separate Sites for Parathyroid Hormone and VasopressinScience, 1968