Zeng Fanyi, Ren Zhao-Rui, Huang Shang-Zhi, Kalf Margot, Mommersteeg Monique, Smit Maarten, White Stefan, Jin Chun-Lian, Xu Miao, Zhou Da-Wen, Yan Jing-Bin, Chen Mei-Jue, van Beuningen Rinie, Huang Shu-Zhen, den Dunnen Johan, Zeng Yi-Tao, Wu Ying
Shanghai Institute of Medical Genetics, Shanghai Children's Hospital, Shanghai Jiao Tong University School of Medicine, PR China.
Hum Mutat. 2008 Jan;29(1):190-7. doi: 10.1002/humu.20613.
Multiplex ligation-dependent probe amplification (MLPA) is widely used to screen genes of interest for deletions and duplications. Since MLPA is usually based on size-separation of the amplification products, the maximum number of target sequences that can be screened in parallel is usually limited to approximately 40. We report the design of a robust array-based MLPA format that uses amplification products of essentially uniform size (100-120 bp) and distinguishes between them by virtue of incorporated tag sequences. We were thus able to increase probe complexity to 124, with very uniform product yields and signals that have a low coefficient of variance. The assay designed was used to screen the largest set studied so far (249 patients) of unrelated Duchenne muscular dystrophy (DMD) cases from the Chinese population. In a blind study we correctly assigned 98% of the genotypes and detected rearrangements in 181 cases (73%); i.e., 163 deletions (65%), 13 duplications (5%), and five complex rearrangements (2%). Although this value is significantly higher for Chinese patients than previously reported, it is similar to that found for other populations. The location of the rearrangements (76% in the major deletion hotspot) is also in agreement with other findings. The 96-well flow-through microarray system used in this research provides high-throughput and speed; hybridization can be completed in 5 to 30 minutes. Since array processing and data analysis are fully automated, array-MLPA should be easy to implement in a standard diagnostic laboratory. The universal array can be used to analyze any tag-modified MLPA probe set.
多重连接依赖探针扩增(MLPA)被广泛用于筛选感兴趣基因的缺失和重复情况。由于MLPA通常基于扩增产物的大小分离,因此可并行筛选的靶序列最大数量通常限制在约40个。我们报告了一种基于芯片的稳健MLPA形式的设计,该形式使用大小基本一致(100 - 120 bp)的扩增产物,并通过掺入的标签序列对它们进行区分。因此,我们能够将探针复杂度提高到124,产物产量非常均匀,信号的变异系数很低。所设计的检测方法用于筛查迄今为止研究的来自中国人群的最大一组(249例)无关杜氏肌营养不良(DMD)病例。在一项盲法研究中,我们正确确定了98%的基因型,并在181例病例(73%)中检测到重排;即163例缺失(65%)、13例重复(5%)和5例复杂重排(2%)。尽管中国患者的这一数值明显高于先前报道,但与其他人群的数值相似。重排的位置(76%在主要缺失热点区域)也与其他研究结果一致。本研究中使用的96孔流通式微阵列系统提供了高通量和速度;杂交可在5至30分钟内完成。由于芯片处理和数据分析完全自动化,基于芯片的MLPA应该易于在标准诊断实验室中实施。通用芯片可用于分析任何经标签修饰的MLPA探针组。