Hayashi Shin, Honda Shozo, Minaguchi Maki, Makita Yoshio, Okamoto Nobuhiko, Kosaki Rika, Okuyama Torayuki, Imoto Issei, Mizutani Shuki, Inazawa Johji
Department of Molecular Cytogenetics, Medical Research Institute and School of Biomedical Science, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.
Department of Pediatric and Developmental Biology, Tokyo Medical and Dental University, 1-5-45 Yushima Bunkyo-ku, Tokyo, 113-8510, Japan.
J Hum Genet. 2007;52(5):397-405. doi: 10.1007/s10038-007-0127-4. Epub 2007 Apr 4.
The human chromosome X is closely associated with congenital disorders and mental retardation (MR), because it contains a significantly higher number of genes than estimated from the proportion in the human genome. We constructed a high-density and high-resolution human chromosome X array (X-tiling array) for comparative genomic hybridization (CGH). The array contains a total of 1,001 bacterial artificial chromosome (BACs) throughout chromosome X except pseudoautosomal regions and two BACs specific for Y. In four hybridizations using DNA samples from healthy males, the ratio of each spotted DNA was scattered between -3SD and 3SD, corresponding to a log(2) ratio of -0.35 and 0.35, respectively. Using DNA samples from patients with known congenital disorders, our X-tiling array was proven to discriminate one-copy losses and gains together with their physical sizes, and also to estimate the percentage of a mosaicism in a patient with mos 45,X[13]/46,X,r(X)[7]. Furthermore, array-CGH in a patient with atypical Schinzel-Giedion syndrome disclosed a 1.1-Mb duplication at Xq22.3 including a part of the IL1RAPL2 gene as a likely causative aberration. The results indicate our in-house X-tiling array to be useful for the identification of cryptic copy-number aberrations containing novel genes responsible for diseases such as congenital disorders and X-linked MR.
人类X染色体与先天性疾病和智力迟钝(MR)密切相关,因为它所含基因数量比根据其在人类基因组中的比例所估计的要多得多。我们构建了一种用于比较基因组杂交(CGH)的高密度、高分辨率人类X染色体阵列(X平铺阵列)。该阵列在整个X染色体上(除假常染色体区域外)共包含1001个细菌人工染色体(BAC)以及两个Y染色体特异性BAC。在使用健康男性DNA样本进行的四次杂交中,每个点样DNA的比率分布在-3SD至3SD之间,分别对应于log(2)比率为-0.35和0.35。使用已知先天性疾病患者的DNA样本,我们的X平铺阵列被证明能够区分单拷贝缺失和增益及其物理大小,还能估计45,X[13]/46,X,r(X)[7]嵌合体患者的嵌合率。此外,对一名非典型辛泽尔-吉迪恩综合征患者进行的阵列CGH分析揭示了Xq22.3处有一个1.1-Mb的重复,其中包括IL1RAPL2基因的一部分,这可能是致病畸变。结果表明,我们自制的X平铺阵列可用于识别包含导致先天性疾病和X连锁MR等疾病的新基因的隐匿性拷贝数畸变。