Takeda K, Yokoyama M, Hashimoto K, Hiromatsu Y, Yamanaka H, Shimizu T, Sasaki M
Department of Clinical Laboratory Medicine, Kochi Medical School, Japan.
Endocr J. 1997 Aug;44(4):621-5. doi: 10.1507/endocrj.44.621.
The identification of unique point mutations in patients with pseudohypoparathyroidism (PHP) with Albright's hereditary osteodystrophy (AHO) in different ethnic backgrounds has proved that defects within the Gs alpha gene account for Gs alpha deficiency in those patients. To search a mutation hot spot of the Gs alpha gene in Japanese patients, we have screened exons 2-13 of the Gs alpha gene for mutations in three unrelated Japanese PHP patients with AHO. We could find no abnormalities by denaturing gradient gel electrophoresis and no mutations of sequencing of exon 7 in these subjects. This suggests that mutations in exon 7 of the Gs alpha gene may not be a common cause of PHP with AHO in Japanese.
在不同种族背景的伴有Albright遗传性骨营养不良(AHO)的假性甲状旁腺功能减退症(PHP)患者中鉴定独特的点突变,已证明Gsα基因内的缺陷是这些患者Gsα缺乏的原因。为了寻找日本患者中Gsα基因的突变热点,我们对3名不相关的伴有AHO的日本PHP患者的Gsα基因外显子2 - 13进行了突变筛查。通过变性梯度凝胶电泳,我们未发现异常,且这些受试者的外显子7测序也未发现突变。这表明Gsα基因外显子7的突变可能不是日本伴有AHO的PHP的常见病因。