Buckle V J, Higgs D R, Wilkie A O, Super M, Weatherall D J
Nuffield Department of Clinical Medicine, John Radcliffe Hospital, Oxford.
J Med Genet. 1988 Dec;25(12):847-9. doi: 10.1136/jmg.25.12.847.
A female child with alpha thalassaemia trait, moderate mental retardation, and dysmorphic features has inherited an abnormal chromosome 16 complement as a result of the unbalanced segregation of a maternal balanced translocation. Cytogenetic analysis indicates that the patient is monosomic for 16p13.3----pter and trisomic for 10q26.13----qter. DNA studies show that the patient has not inherited either maternal alpha globin allele. This accounts for the alpha thalassaemia trait in the child and places the human alpha globin complex in band 16p13.3----pter.
一名患有α地中海贫血特征、中度智力发育迟缓且有畸形特征的女童,由于母亲平衡易位的不平衡分离而继承了异常的16号染色体组。细胞遗传学分析表明,该患者16p13.3至染色体末端为单体型,10q26.13至染色体末端为三体型。DNA研究显示,该患者未继承任何一个母亲的α珠蛋白等位基因。这解释了患儿的α地中海贫血特征,并将人类α珠蛋白复合体定位于16p13.3至染色体末端区域。