Simpson J L, Lebeau M M
Am J Obstet Gynecol. 1981 Dec 15;141(8):930-40. doi: 10.1016/s0002-9378(16)32685-0.
Correlation of clinical features with cytogenetic abnormalities for individuals showing deletions of the X short arm (Xp) or the X long arm (Xq) indicate the following: (1) both Xp and Xq are necessary to assure normal ovarian development, although (2) persisting ovarian function is not infrequently associated with either (del(X)(p11) or del(Xq)(13,21,22, or 24). (3) Ovarian determinants on Xp are localized to region Xp11, but determinants on Xq cannot be precisely localized. (4) Both Xp and Xq contain statural determinants, the former localized to region Xp21 leads to Xpter. Both cell generation time and phases of the cell cycle were studied to test the hypothesis that the short stature, intrauterine growth retardation, and high embryonic lethality of 45,X can be explained on the basis of intrinsic retardation of cell division (i.e., prolonged cell cycle). Cell generation times of four 45,X fibroblast lines were significantly longer than those of for normal diploid lines, a difference accounted for by a prolonged S phase. 46,X,del(X)(p11), 46,X,del(X)(q13), and 46,X,del(X)(q22) lines also showed increased cell generation times when compared to 46,XX lines.
对于显示X短臂(Xp)或X长臂(Xq)缺失的个体,临床特征与细胞遗传学异常之间的相关性表明如下:(1)Xp和Xq对于确保正常卵巢发育都是必需的,尽管(2)持续的卵巢功能并不罕见地与del(X)(p11)或del(Xq)(13、21、22或24)相关。(3)Xp上的卵巢决定因素定位于Xp11区域,但Xq上的决定因素无法精确定位。(4)Xp和Xq都包含身高决定因素,前者定位于Xp21区域直至Xpter。研究了细胞生成时间和细胞周期的各个阶段,以检验以下假设:45,X个体的身材矮小、宫内生长迟缓以及高胚胎致死率可以基于细胞分裂的内在迟缓(即延长的细胞周期)来解释。四条45,X成纤维细胞系的细胞生成时间明显长于正常二倍体细胞系,这种差异是由S期延长所致。46,X,del(X)(p11)、46,X,del(X)(q13)和46,X,del(X)(q22)细胞系与46,XX细胞系相比,其细胞生成时间也有所增加。