Sutherland G R, Haan E A, Kremer E, Lynch M, Pritchard M, Yu S, Richards R I
Department of Cytogenetics and Molecular Genetics, Adelaide Children's Hospital, Australia.
Lancet. 1991 Aug 3;338(8762):289-92. doi: 10.1016/0140-6736(91)90426-p.
Fragile X syndrome, associated with the fragile X chromosome, is the most common cause of familial mental retardation. The condition is characterised by a heritable DNA sequence that consists of an abnormal number of CCG repeats, and which is unstable in both mitosis and meiosis. We suggest that such heritable unstable DNA sequences could be present in other parts of the genome and that these might explain a number of genetic events that are not well understood in terms of classic genetic mechanisms. Such poorly explained observations include anticipation, incomplete penetrance, variable expression, and possibly imprinting, variegation, and multifactorial inheritance.
与脆性X染色体相关的脆性X综合征是家族性智力迟钝最常见的病因。该病症的特征是一种可遗传的DNA序列,其CCG重复序列数量异常,并且在有丝分裂和减数分裂中均不稳定。我们认为,这种可遗传的不稳定DNA序列可能存在于基因组的其他部位,这或许可以解释一些经典遗传机制难以理解的遗传事件。这类解释不充分的现象包括遗传早现、不完全显性、可变表达,可能还包括印记、斑驳现象以及多因素遗传。