Valenzuela D, Han X, Mende U, Fankhauser C, Mashimo H, Huang P, Pfeffer J, Neer E J, Fishman M C
Laboratory of Developmental Biology and Cardiovascular Research Center, Massachusetts General Hospital, Charlestown 02129, USA.
Proc Natl Acad Sci U S A. 1997 Mar 4;94(5):1727-32. doi: 10.1073/pnas.94.5.1727.
Heterotrimeric G proteins, composed of G alpha and G betagamma subunits, transmit signals from cell surface receptors to cellular effector enzymes and ion channels. The G alpha(o) protein is the most abundant G alpha subtype in the nervous system, but it is also found in the heart. Its function is not completely known, although it is required for regulation of N-type Ca2+ channels in GH3 cells and also interacts with GAP43, a major protein in growth cones, suggesting a role in neuronal pathfinding. To analyze the function of G alpha(o), we have generated mice lacking both isoforms of G alpha(o) by homologous recombination. Surprisingly, the nervous system is grossly intact, despite the fact that G alpha(o) makes up 0.2-0.5% of brain particulate protein and 10% of the growth cone membrane. The G alpha(o)-/- mice do suffer tremors and occasional seizures, but there is no obvious histologic abnormality in the nervous system. In contrast, G alpha(o)-/- mice have a clear and specific defect in ion channel regulation in the heart. Normal muscarinic regulation of L-type calcium channels in ventricular myocytes is absent in the mutant mice. The L-type calcium channel responds normally to isoproterenol, but there is no evident muscarinic inhibition. Muscarinic regulation of atrial K+ channels is normal, as is the electrocardiogram. The levels of other G alpha subunits (G alpha(s), G alpha(q), and G alpha(i)) are unchanged in the hearts of G alpha(o)-/- mice, but the amount of G betagamma is decreased. Whichever subunit, G alpha(o) or G betagamma, carries the signal forward, these studies show that muscarinic inhibition of L-type Ca2+ channels requires coupling of the muscarinic receptor to G alpha(o). Other cardiac G alpha subunits cannot substitute.
异源三聚体G蛋白由Gα和Gβγ亚基组成,可将信号从细胞表面受体传递至细胞效应酶和离子通道。Gα(o)蛋白是神经系统中含量最丰富的Gα亚型,但在心脏中也有发现。尽管它对于调节GH3细胞中的N型Ca2+通道是必需的,并且还与生长锥中的主要蛋白GAP43相互作用,提示其在神经元寻路中发挥作用,但其功能尚未完全明确。为了分析Gα(o)的功能,我们通过同源重组构建了缺失Gα(o)两种同工型的小鼠。令人惊讶的是,尽管Gα(o)占脑颗粒蛋白的0.2 - 0.5%以及生长锥膜的10%,但神经系统大体上是完整的。Gα(o)-/-小鼠确实会出现震颤和偶尔的癫痫发作,但神经系统没有明显的组织学异常。相比之下,Gα(o)-/-小鼠在心脏离子通道调节方面存在明显且特定的缺陷。突变小鼠的心室肌细胞中L型钙通道的正常毒蕈碱调节缺失。L型钙通道对异丙肾上腺素反应正常,但没有明显的毒蕈碱抑制作用。心房K+通道的毒蕈碱调节正常,心电图也正常。Gα(o)-/-小鼠心脏中其他Gα亚基(Gα(s)、Gα(q)和Gα(i))的水平没有变化,但Gβγ的量减少。无论向前传递信号的是Gα(o)还是Gβγ亚基,这些研究表明L型Ca2+通道的毒蕈碱抑制需要毒蕈碱受体与Gα(o)偶联。其他心脏Gα亚基不能替代。