Endocrine Unit, Departments of Medicine and Pediatrics, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA.
J Biol Chem. 2011 Jan 14;286(2):1618-26. doi: 10.1074/jbc.M110.198416. Epub 2010 Nov 3.
The parathyroid hormone (PTH)/PTH-related peptide (PTHrP) receptor (PTHR1) in cells of the renal proximal tubule mediates the reduction in membrane expression of the sodium-dependent P(i) co-transporters, NPT2a and NPT2c, and thus suppresses the re-uptake of P(i) from the filtrate. In most cell types, the liganded PTHR1 activates Gα(S)/adenylyl cyclase/cAMP/PKA (cAMP/PKA) and Gα(q/11)/phospholipase C/phosphatidylinositol 1,4,5-trisphosphate (IP(3))/Ca(2+)/PKC (IP(3)/PKC) signaling pathways, but the relative roles of each pathway in mediating renal regulation P(i) transport remain uncertain. We therefore explored the signaling mechanisms involved in PTH-dependent regulation of NPT2a function using potent, long-acting PTH analogs, M-PTH(1-28) (where M = Ala(1,12), Aib(3), Gln(10), Har(11), Trp(14), and Arg(19)) and its position 1-modified variant, Trp(1)-M-PTH(1-28), designed to be phospholipase C-deficient. In cell-based assays, both M-PTH(1-28) and Trp(1)-M-PTH(1-28) exhibited potent and prolonged cAMP responses, whereas only M-PTH(1-28) was effective in inducing IP(3) and intracellular calcium responses. In opossum kidney cells, a clonal cell line in which the PTHR1 and NPT2a are endogenously expressed, M-PTH(1-28) and Trp(1)-M-PTH(1-28) each induced reductions in (32)P uptake, and these responses persisted for more than 24 h after ligand wash-out, whereas that of PTH(1-34) was terminated by 4 h. When injected into wild-type mice, both M-modified PTH analogs induced prolonged reductions in blood P(i) levels and commensurate reductions in NPT2a expression in the renal brush border membrane. Our findings suggest that the acute down-regulation of NPT2a expression by PTH ligands involves mainly the cAMP/PKA signaling pathway and are thus consistent with the elevated blood P(i) levels seen in pseudohypoparathyroid patients, in whom Gα(s)-mediated signaling in renal proximal tubule cells is defective.
甲状旁腺激素(PTH)/甲状旁腺激素相关肽(PTHrP)受体(PTHR1)在肾近端小管细胞中介导钠依赖性磷酸(Pi)共转运体 NPT2a 和 NPT2c 的膜表达减少,从而抑制 Pi 从滤液中的重吸收。在大多数细胞类型中,配体结合的 PTHR1 激活 Gα(S)/腺苷酸环化酶/cAMP/PKA(cAMP/PKA)和 Gα(q/11)/磷脂酶 C/磷酸肌醇 1,4,5-三磷酸(IP(3))/Ca(2+)/PKC(IP(3)/PKC)信号通路,但每种通路在介导肾脏调节 Pi 转运中的相对作用仍不确定。因此,我们使用强效、长效 PTH 类似物 M-PTH(1-28)(其中 M = Ala(1,12)、Aib(3)、Gln(10)、Har(11)、Trp(14)和 Arg(19))及其位置 1 修饰变体 Trp(1)-M-PTH(1-28)探索了 PTH 依赖性调节 NPT2a 功能的信号机制,该变体设计为缺乏磷脂酶 C。在基于细胞的测定中,M-PTH(1-28)和 Trp(1)-M-PTH(1-28)均表现出强大且持久的 cAMP 反应,而只有 M-PTH(1-28)能够有效诱导 IP(3)和细胞内钙反应。在美洲负鼠肾细胞(一种克隆细胞系,其中 PTHR1 和 NPT2a 内源性表达)中,M-PTH(1-28)和 Trp(1)-M-PTH(1-28)均诱导 (32)P 摄取减少,并且这些反应在配体洗脱后持续超过 24 小时,而 PTH(1-34)的反应在 4 小时后终止。当注射到野生型小鼠中时,两种 M 修饰的 PTH 类似物均诱导血液 Pi 水平的长期降低,并伴随肾脏刷状缘膜 NPT2a 表达的降低。我们的发现表明,PTH 配体对 NPT2a 表达的急性下调主要涉及 cAMP/PKA 信号通路,这与假性甲状旁腺功能减退症患者中观察到的升高的血液 Pi 水平一致,在假性甲状旁腺功能减退症患者中,肾近端小管细胞中的 Gα(s)-介导信号传导存在缺陷。