Brown E M, Pollak M, Hebert S C
Department of Medicine, Brigham and Women's Hospital, Boston, MA 02186.
Am J Kidney Dis. 1995 Mar;25(3):506-13. doi: 10.1016/0272-6386(95)90118-3.
The ability of the parathyroid cell to sense minute fluctuations in the extracellular ionized calcium concentration (Ca2+ o) is essential for maintaining mineral ion homeostasis. However, the mechanism(s) through which the parathyroid cell and other cells recognize and respond to changes in Ca2+ o has remained unclear. We recently isolated a cDNA encoding a Ca2+ o-sensing receptor from bovine parathyroid using expression cloning in Xenopus laevis oocytes. The receptor shows pharmacologic properties that are almost identical to those of the receptor on the parathyroid cell and, like the latter, stimulates phospholipase C in a G-protein-dependent manner. The amino acid sequence of the cloned receptor deduced from this cDNA predicts a protein with a molecular mass of 121 kd, which has three principal structural domains. The first is a 613 amino acid, putatively extracellular amino terminus which has several regions rich in acidic amino acids that may potentially be involved in binding Ca2+ and other polycationic agonists. The second comprises seven membrane-spanning segments that are characteristic of the superfamily of G-protein-coupled receptors, and the third is a 222 amino acid cytoplasmic tail. Transcripts for this Ca2+ o-sensing receptor are present in the parathyroid as well as in the kidney, thyroid, and brain. We next investigated the hypercalcemic disorders, familial hypocalciuric hypercalcemia and neonatal severe hyperparathyroidism, as possible examples of inherited abnormalities in this Ca2+ o-sensing receptor, since both disorders show abnormal Ca2+ o-sensing and/or handling in the kidney and parathyroid.(ABSTRACT TRUNCATED AT 250 WORDS)
甲状旁腺细胞感知细胞外离子钙浓度(Ca2+o)微小波动的能力对于维持矿物质离子稳态至关重要。然而,甲状旁腺细胞和其他细胞识别并响应Ca2+o变化的机制仍不清楚。我们最近利用非洲爪蟾卵母细胞中的表达克隆技术,从牛甲状旁腺中分离出一种编码Ca2+o传感受体的cDNA。该受体显示出与甲状旁腺细胞上受体几乎相同的药理学特性,并且与后者一样,以G蛋白依赖性方式刺激磷脂酶C。从该cDNA推导的克隆受体的氨基酸序列预测其为一种分子量为121kd的蛋白质,具有三个主要结构域。第一个是一个613个氨基酸的假定细胞外氨基末端,有几个富含酸性氨基酸的区域,可能参与结合Ca2+和其他多阳离子激动剂。第二个包含七个跨膜片段,这是G蛋白偶联受体超家族的特征,第三个是一个222个氨基酸的胞质尾巴。这种Ca2+o传感受体的转录本存在于甲状旁腺以及肾脏、甲状腺和大脑中。接下来,我们研究了高钙血症疾病、家族性低钙血症性高钙血症和新生儿重症甲状旁腺功能亢进,作为这种Ca2+o传感受体遗传性异常的可能例子,因为这两种疾病在肾脏和甲状旁腺中均表现出异常的Ca2+o传感和/或处理。(摘要截短于250字)