The Laboratory of Genetics and Metabolism, Hunan Children's Research Institute (HCRI), Hunan Children's Hospital, The Paediatric Academy of University of South China, Changsha 410008, China.
Biomed Res Int. 2013;2013:689756. doi: 10.1155/2013/689756. Epub 2013 Aug 13.
Piebaldism is a rare autosomal dominant disorder of melanocyte development, which is mostly caused by KIT gene. The key characteristics of piebaldism include localized poliosis, congenital leukoderma, and other variable manifestations. The previous study has illustrated that the homogeneous MC1R (a gene which is associated with the hair color) variant (p.I120T) coordinating with KIT mutation may lead to auburn hair color and piebaldism. In this study, we have investigated a Chinese family with piebaldism and auburn hair color; the mutation screening of KIT and MC1R genes identified that only a splicing mutation (c. 2484+1G>A) of KIT gene cosegregated with the auburn hair color and piebaldism. The data of this study and others suggests that the KIT mutation may causes of the auburn hair color in the piebaldism patients.
斑驳病是一种罕见的常染色体显性黑色素细胞发育障碍,主要由 KIT 基因突变引起。斑驳病的主要特征包括局部白发、先天性白癜和其他可变表现。先前的研究表明,与头发颜色相关的 MC1R(一种基因)的同质变体(p.I120T)与 KIT 突变协同作用可能导致淡褐色头发和斑驳病。在这项研究中,我们调查了一个具有斑驳病和淡褐色头发颜色的中国家庭;KIT 和 MC1R 基因突变的筛选发现,只有 KIT 基因的剪接突变(c.2484+1G>A)与淡褐色头发颜色和斑驳病共分离。本研究和其他研究的数据表明,KIT 突变可能是斑驳病患者淡褐色头发的原因。