Ting S S, Ziegler J, Leigh D, Kemp A, Lindeman R
Department of Immunology/Allergy, Sydney Children's Hospital, Australia.
J Pediatr. 1998 Oct;133(4):575-6. doi: 10.1016/s0022-3476(98)70073-3.
We performed gamma c gene analysis of a boy with severe combined immunodeficiency whose brother died of the same condition. A base pair deletion in exon 6 was found, which was absent in his mother, indicating maternal mosaicism with important implications for genetic counseling and demonstrating the importance of mutation analysis in boys with severe combined immunodeficiency.
我们对一名患有严重联合免疫缺陷的男孩进行了γc基因分析,该男孩的哥哥死于同样的病症。在外显子6中发现了一个碱基对缺失,而他的母亲没有这个缺失,这表明存在母系嵌合体,对遗传咨询具有重要意义,并证明了对患有严重联合免疫缺陷的男孩进行突变分析的重要性。