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

R. W. Downs, M. A. Levine, M. K. Drezner, W. M. Burch, A. M. Spiegel

Research output: Contribution to journalArticle

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-γ-S). In cholate extracts, the G unit activity was 37 and 48% of control with fluoride or GTP-γ-S, respectively. Cholera toxin-dependent incorporation of [ 32P]ADP-ribose into the 42,000-M(r) 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.

Original languageEnglish (US)
Pages (from-to)231-235
Number of pages5
JournalJournal of Clinical Investigation
Volume71
Issue number2
StatePublished - 1983
Externally publishedYes

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ASJC Scopus subject areas

  • Medicine(all)

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Downs, R. W., Levine, M. A., Drezner, M. K., Burch, W. M., & Spiegel, A. M. (1983). Deficient adenylate cyclase regulatory protein in renal membranes from a patient with pseudohypoparathyroidism. Journal of Clinical Investigation, 71(2), 231-235.