Upadhyaya M, Roberts S H, Maynard J, Sorour E, Thompson P W, Vaughan M, Wilkie A O, Hughes H E
Institute of Medical Genetics, University Hospital of Wales Cardiff, UK.
J Med Genet. 1996 Feb;33(2):148-52. doi: 10.1136/jmg.33.2.148.
We report the first visible cytogenetic deletion involving the NF1 gene in a patient with sporadic neurofibromatosis, dysmorphic features, and marked developmental delay. The combined evidence of molecular and cytogenetic techniques based on dosage reduction, hemizygosity for microsatellite markers, high resolution G banding, and FISH analysis, predicts this deletion to be approximately 7 Mb in size. Our findings highlight the importance of conducting a detailed cytogenetic and FISH analysis in patients with NF1 who have additional dysmorphic features or particularly severe learning difficulties.
我们报告了首例在患有散发性神经纤维瘤病、畸形特征和明显发育迟缓的患者中涉及NF1基因的可见细胞遗传学缺失。基于剂量减少、微卫星标记半合子状态、高分辨率G显带和荧光原位杂交(FISH)分析的分子和细胞遗传学技术的综合证据表明,该缺失大小约为7 Mb。我们的研究结果突出了对具有额外畸形特征或特别严重学习困难的NF1患者进行详细细胞遗传学和FISH分析的重要性。