Shaw-Smith C, Redon R, Rickman L, Rio M, Willatt L, Fiegler H, Firth H, Sanlaville D, Winter R, Colleaux L, Bobrow M, Carter N P
University of Cambridge Department of Medical Genetics, Addenbrooke's Hospital, Hills Road, Cambridge, UK.
J Med Genet. 2004 Apr;41(4):241-8. doi: 10.1136/jmg.2003.017731.
The underlying causes of learning disability and dysmorphic features in many patients remain unidentified despite extensive investigation. Routine karyotype analysis is not sensitive enough to detect subtle chromosome rearrangements (less than 5 Mb). The presence of subtle DNA copy number changes was investigated by array-CGH in 50 patients with learning disability and dysmorphism, employing a DNA microarray constructed from large insert clones spaced at approximately 1 Mb intervals across the genome. Twelve copy number abnormalities were identified in 12 patients (24% of the total): seven deletions (six apparently de novo and one inherited from a phenotypically normal parent) and five duplications (one de novo and four inherited from phenotypically normal parents). Altered segments ranged in size from those involving a single clone to regions as large as 14 Mb. No recurrent deletion or duplication was identified within this cohort of patients. On the basis of these results, we anticipate that array-CGH will become a routine method of genome-wide screening for imbalanced rearrangements in children with learning disability.
尽管进行了广泛的调查,但许多患者学习障碍和畸形特征的潜在原因仍未明确。常规核型分析对检测细微的染色体重排(小于5 Mb)不够敏感。采用一种由全基因组间隔约1 Mb的大插入片段克隆构建的DNA微阵列,通过比较基因组杂交芯片技术(array-CGH)对50例有学习障碍和畸形的患者进行了细微DNA拷贝数变化的检测。在12例患者(占总数的24%)中发现了12个拷贝数异常:7个缺失(6个明显为新发突变,1个从表型正常的父母遗传而来)和5个重复(1个新发突变,4个从表型正常的父母遗传而来)。改变的片段大小从涉及单个克隆到高达14 Mb的区域不等。在这组患者中未发现复发性缺失或重复。基于这些结果,我们预计比较基因组杂交芯片技术将成为对有学习障碍儿童进行全基因组不平衡重排筛查的常规方法。