Deficient adenylate cyclase regulatory protein in renal membranes from a patient with pseudohypoparathyroidism.

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Downs, RW; Levine, MA; Drezner, MK; Burch, WM; Spiegel, AM

Published Date

  • February 1, 1983

Published In

Volume / Issue

  • 71 / 2

Start / End Page

  • 231 - 235

PubMed ID

  • 6822662

Pubmed Central ID

  • 6822662

International Standard Serial Number (ISSN)

  • 0021-9738

Digital Object Identifier (DOI)

  • 10.1172/jci110763

Language

  • eng

Conference Location

  • United States