Kałuzewski B, Jakubowski L, Debiec-Rychter M, Grzeschik K H, Limon J, Gibas Z
Department of Medical Genetics, Medical Academy of Lódź, Poland.
Am J Med Genet. 1988 Nov;31(3):489-503. doi: 10.1002/ajmg.1320310303.
Two cases of a nonfluorescent Y (Ynf) chromosome were diagnosed: one in a male, the other in a female. Both had similar complex mosaic chromosome constitutions with a 45,X cell line. DNA studies were applied in both cases for verification of the cytogenetic diagnosis. The results on the two patients were compared with data obtained from seven healthy men (46,XY), three healthy women (46,XX), two females with 46,XY karyotype, and from cell lines with 49,XXXXY and 48,XXXX chromosome constitution. The highly repetitive Y-specific DNA sequences located in the heterochromatic region of the long arm were absent in these patients. Differences in the composition of the euchromatic part of the Y chromosome were demonstrable in both patients. The highly repetitive Y-specific DNA sequences located in the heterochromatic region of the long arm were absent in these patients. Differences in the composition of the euchromatic part of the Y chromosome were demonstrable in both patients. The suggestion that the Ynf chromosome originates from a dicentric Y chromosome cannot be accepted as a complete explanation of the phenomenon, as it probably involves more complex molecular alterations of the abnormal Y chromosome. The presence of Ynf is associated with the presence of a 45,X cell line more often than in cases of simple Y chromosome deletions with the breakpoint localized in or below the Y euchromatin/heterochromatin junction.
诊断出两例非荧光Y(Ynf)染色体病例:一例为男性,另一例为女性。两者都具有相似的复杂嵌合染色体组成,伴有45,X细胞系。对这两例病例均进行了DNA研究,以验证细胞遗传学诊断。将这两名患者的结果与从七名健康男性(46,XY)、三名健康女性(46,XX)、两名46,XY核型女性以及具有49,XXXXY和48,XXXX染色体组成的细胞系中获得的数据进行了比较。这些患者的Y染色体长臂异染色质区域中高度重复的Y特异性DNA序列缺失。在两名患者中均证实了Y染色体常染色质部分组成的差异。这些患者的Y染色体长臂异染色质区域中高度重复的Y特异性DNA序列缺失。在两名患者中均证实了Y染色体常染色质部分组成的差异。Ynf染色体起源于双着丝粒Y染色体的观点不能被完全接受为对该现象的解释,因为这可能涉及异常Y染色体更复杂的分子改变。与简单Y染色体缺失且断点位于Y常染色质/异染色质交界处或其下方的情况相比,Ynf的存在与45,X细胞系的存在关联更为常见。