{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T01:13:31Z","timestamp":1782695611594,"version":"3.54.5"},"reference-count":41,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[1996,1,1]],"date-time":"1996-01-01T00:00:00Z","timestamp":820454400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Medical Research Society of Canada","award":["MI I0900"],"award-info":[{"award-number":["MI I0900"]}]},{"DOI":"10.13039\/100000002","name":"National Institutes of health","doi-asserted-by":"crossref","award":["DK3428I"],"award-info":[{"award-number":["DK3428I"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1996,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Pseudohypoparathyroidism (PHP) is characterized by a lack of response to parathyroid hormone (PTH); however, normal skeletal responsiveness to PTH in some patients with PHP type la was previously suggested on the basis of clinical observations. To test this hypothesis, we measured cyclic adenosine monophosphate (cAMP) production in response to various agonists in bone-derived osteoblast-like (OBL) cells from trabecular explants obtained from an iliac crest biopsy of a 25-year-old woman with PHP. The patient was proved to have PHP type la on the basis of Albright's hereditary osteodystrophy and decreased activity of stimulatory guanine nucleotide-binding protein (Gs) in erythrocytes. Responsiveness of the patient's OBL cells was compared with OBL cells from eight subjects aged 18\u201339 years who had no evidence of metabolic bone disease. OBL cells from the patient responded to the following agonists (expressed in multiples of elevation of cAMP, stimulated\/basal, mean \u00b1 SE, n = 3): PTH, 3.8 \u00b1 0.3; forskolin, 8.2 \u00b1 0.2; and cholera toxin, 56.8 \u00b1 10.0. These responses were not significantly different from those of control OBL cells: PTH, 4.5 \u00b1 1.1 (range 2.4\u20137.5); forskolin, 7.7 \u00b1 1.4; and cholera toxin, 57.9 \u00b1 16.2. The normal cholera toxin response indicated the presence of functional Gs. Bone cells from patients with PHP type la may exhibit a normal PTH receptor-coupled adenylyl cyclase system in vitro despite clinical evidence of impaired hormone-responsive adenylyl cyclase in other tissues, including the kidney. Skeletal responsiveness to PTH may explain the long periods of spontaneous normocalcemia observed in this patient.<\/jats:p>","DOI":"10.1002\/jbmr.5650110103","type":"journal-article","created":{"date-parts":[[2010,2,3]],"date-time":"2010-02-03T15:27:52Z","timestamp":1265210872000},"page":"8-14","source":"Crossref","is-referenced-by-count":37,"title":["Normal parathyroid hormone responsiveness of bone-derived cells from a patient with pseudohypoparathyroidism"],"prefix":"10.1093","volume":"11","author":[{"given":"Sophia","family":"Ish-Shalom","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Rambam Medical Center and The Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Letitia G.","family":"Rao","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Toronto and Research Institute, Queen Elizabeth Hospital, Toronto, Ontario, Canada"},{"name":"Division of Endocrinology and Metabolism, St. Michael's Hospital, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Levine","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism, Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald","family":"Fraser","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Toronto and Research Institute, Queen Elizabeth Hospital, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang W.","family":"Kooh","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Toronto and Research Institute, Queen Elizabeth Hospital, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert 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