TY - JOUR
T1 - Deficiency of hormone receptor-adenylate cyclase coupling protein
T2 - Basis for hormone resistance in pseudohypoparathyroidism
AU - Spiegel, A. M.
AU - Levine, M. A.
AU - Aurbach, G. D.
N1 - Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 1982
Y1 - 1982
N2 - Pseudohypoparathyroidism is an inherited disorder associated with resistance to the action of several hormones, including parathyroid hormone, thyroid-stimulating hormone, follicle-stimulating hormone, and luteinizing hormone. The disorders described under this designation are heterogeneous in regard to the underlying genetic defects, the phenotypic manifestations, and the severity of the defects in hormone action. The majority of affected individuals who also have the characteristic skeletal changes (heredity osteodystrophy) have a defect in the guanine nucleotide regulatory protein (G protein) that is essential for coupling certain cell-surface hormone receptors to the adenylate cyclase system. This defect is probably the cause for resistance to the action of multiple hormones. In the remaining patients the cause for hormone resistance has not been identified.
AB - Pseudohypoparathyroidism is an inherited disorder associated with resistance to the action of several hormones, including parathyroid hormone, thyroid-stimulating hormone, follicle-stimulating hormone, and luteinizing hormone. The disorders described under this designation are heterogeneous in regard to the underlying genetic defects, the phenotypic manifestations, and the severity of the defects in hormone action. The majority of affected individuals who also have the characteristic skeletal changes (heredity osteodystrophy) have a defect in the guanine nucleotide regulatory protein (G protein) that is essential for coupling certain cell-surface hormone receptors to the adenylate cyclase system. This defect is probably the cause for resistance to the action of multiple hormones. In the remaining patients the cause for hormone resistance has not been identified.
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U2 - 10.1152/ajpendo.1982.243.1.e37
DO - 10.1152/ajpendo.1982.243.1.e37
M3 - Article
C2 - 6283911
AN - SCOPUS:0020158214
SN - 0193-1849
VL - 6
SP - E37-E42
JO - American Journal of Physiology - Endocrinology and Metabolism
JF - American Journal of Physiology - Endocrinology and Metabolism
IS - 1
ER -