Bastida Eizaguirre M, Iturbe Ortiz De Urbina R, Arto Urzainqui M, Ezquerra Larreina R, Escalada San Martín J
Servicios de Pediatría Endocrinología. Hospital Santiago Apóstol. Vitoria.
An Esp Pediatr. 2001 Jun;54(6):598-600.
Studies to detect mutations in the GNAS1 gene were performed in a male patient with features of Albright hereditary osteodystrophy and resistance of target tissues to parathyroid hormone (Pseudohypoparathyroidism Ia). The same investigations were carried out in the patient's mother who showed somatic features of Albright's hereditary osteodystrophy and brachymetacarpia without resistance to parathyroid hormone (Pseudopseudohypoparathyroidism). A point mutation designated c.794GA (R265H) in exon 10 of GNAS1 was identified in DNA from the patient and his mother. This novel mutation in exon 10 of GNA
对一名患有奥尔布赖特遗传性骨营养不良特征且靶组织对甲状旁腺激素有抵抗(假性甲状旁腺功能减退症Ia型)的男性患者进行了检测GNAS1基因突变的研究。对患者的母亲也进行了同样的检查,她表现出奥尔布赖特遗传性骨营养不良的体征和短掌骨,但对甲状旁腺激素无抵抗(假假性甲状旁腺功能减退症)。在患者及其母亲的DNA中鉴定出GNAS1第10外显子中的一个点突变,命名为c.794G>A(R265H)。GNAS第10外显子中的这个新突变