Daniel A, Saville T, Southall D B
J Med Genet. 1979 Aug;16(4):278-84. doi: 10.1136/jmg.16.4.278.
A new dicentric X isochromosome i(X)(pter to q2102 to pter) of similar size to a normal X is described in a girl with gonadal dysgenesis. In this non-mosaic case with an X short arm duplication, most of the stigmata associated with Turner's syndrome were absent. This structure was compared with that of six i(Xq) and three del(X). The del(Xq) structures all possessed a regular sized C band, but in the i(Xq) this was double sized in each case. Phenotypic comparisons are made in the Xq deletions, and some presumptive short arm isochromosomes are reinterpreted as Xq deletions. Incomplete centromeric suppression is suggested as the causal mechanism of mosaicism of sex isochromosomes with 45,X cells, and it is argued that an exchange event between homologoues is an unlikely mechanism to explain sex isochromosome origin.
在一名患有性腺发育不全的女孩中,发现了一条新的双着丝粒X等臂染色体i(X)(pter至q2102至pter),其大小与正常X染色体相似。在这个非嵌合病例中,存在X短臂重复,大多数与特纳综合征相关的体征并不存在。将该结构与6条i(Xq)和3条del(X)的结构进行了比较。del(Xq)结构均具有正常大小的C带,但在i(Xq)中,每种情况下C带大小都是正常的两倍。对Xq缺失进行了表型比较,并将一些推测的短臂等臂染色体重新解释为Xq缺失。不完全着丝粒抑制被认为是性等臂染色体与45,X细胞嵌合的因果机制,并且有人认为同源染色体之间的交换事件不太可能是解释性等臂染色体起源的机制。