Chen Min, Gavrilova Oksana, Liu Jie, Xie Tao, Deng Chuxia, Nguyen Annie T, Nackers Lisa M, Lorenzo Javier, Shen Laura, Weinstein Lee S
Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Proc Natl Acad Sci U S A. 2005 May 17;102(20):7386-91. doi: 10.1073/pnas.0408268102. Epub 2005 May 9.
Gnas is an imprinted gene with multiple gene products resulting from alternative splicing of different first exons onto a common exon 2. These products include stimulatory G protein alpha-subunit (G(s)alpha), the G protein required for receptor-stimulated cAMP production; extralarge G(s)alpha (XLalphas), a paternally expressed G(s)alpha isoform; and neuroendocrine-specific protein (NESP55), a maternally expressed chromogranin-like protein. G(s)alpha undergoes tissue-specific imprinting, being expressed primarily from the maternal allele in certain tissues. Heterozygous mutation of exon 2 on the maternal (E2m-/+) or paternal (E2+/p-) allele results in opposite effects on energy metabolism. E2m-/+ mice are obese and hypometabolic, whereas E2+/p- mice are lean and hypermetabolic. We now studied the effects of G(s)alpha deficiency without disrupting other Gnas gene products by deleting G(s)alpha exon 1 (E1). E1+/p- mice lacked the E2+/p- phenotype and developed obesity and insulin resistance. The lean, hypermetabolic, and insulin-sensitive E2+/p- phenotype appears to result from XLalphas deficiency, whereas loss of paternal-specific G(s)alpha expression in E1+/p- mice leads to an opposite metabolic phenotype. Thus, alternative Gnas gene products have opposing effects on glucose and lipid metabolism. Like E2m-/+ mice, E1m-/+ mice had s.c. edema at birth, presumably due to loss of maternal G(s)alpha expression. However, E1m-/+ mice differed from E2m-/+ mice in other respects, raising the possibility for the presence of other maternal-specific gene products. E1m-/+ mice had more severe obesity and insulin resistance and lower metabolic rate relative to E1+/p- mice. Differences between E1m-/+ and E1+/p- mice presumably result from differential effects on G(s)alpha expression in tissues where G(s)alpha is normally imprinted.
Gnas是一个印记基因,通过不同的第一个外显子选择性剪接至共同的外显子2产生多种基因产物。这些产物包括刺激性G蛋白α亚基(G(s)α),即受体刺激的cAMP产生所需的G蛋白;超大G(s)α(XLαs),一种父本表达的G(s)α异构体;以及神经内分泌特异性蛋白(NESP55),一种母本表达的嗜铬粒蛋白样蛋白。G(s)α经历组织特异性印记,在某些组织中主要从母本等位基因表达。母本(E2m-/+)或父本(E2+/p-)等位基因上外显子2的杂合突变对能量代谢产生相反的影响。E2m-/+小鼠肥胖且代谢低下,而E2+/p-小鼠消瘦且代谢亢进。我们现在通过删除G(s)α外显子1(E1)来研究G(s)α缺乏而不破坏其他Gnas基因产物的影响。E1+/p-小鼠缺乏E2+/p-表型,并出现肥胖和胰岛素抵抗。消瘦、代谢亢进和胰岛素敏感的E2+/p-表型似乎是由于XLαs缺乏导致的,而E1+/p-小鼠中父本特异性G(s)α表达的缺失导致了相反的代谢表型。因此,Gnas基因的不同产物对葡萄糖和脂质代谢有相反的影响。与E2m-/+小鼠一样,E1m-/+小鼠出生时出现皮下水肿,可能是由于母本G(s)α表达缺失。然而,E1m-/+小鼠在其他方面与E2m-/+小鼠不同,这增加了存在其他母本特异性基因产物的可能性。相对于E1+/p-小鼠,E1m-/+小鼠有更严重的肥胖和胰岛素抵抗,且代谢率更低。E1m-/+和E1+/p-小鼠之间的差异可能是由于对G(s)α正常印记的组织中G(s)α表达的不同影响所致。