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一例不同于沃纳综合征的早衰病例中的淋巴细胞增殖和核仁沉降

Lymphocyte proliferation and nucleoid sedimentation in a case of premature aging distinct from Werner's syndrome.

作者信息

Kim M A, Happle R, Traupe H

出版信息

Arch Dermatol Res. 1986;278(4):277-82. doi: 10.1007/BF00407738.

Abstract

Lymphocyte proliferation and nucleoid sedimentation were studied in a patient with premature aging resembling the Werner's syndrome (WS). Onset of patchy brown hyperpigmentations at the age of 9 months permitted distinction from classical WS and suggested a WS-like premature aging disease. By photometric recording of density changes during cell culture, we examined the course of cell proliferation after PHA stimulation over 7 days and compared these results to those obtained in two normal controls. Cultured cells of the patient displayed an aberrant proliferation pattern characterized by continuous growth without an initial reduction phase. The markedly reduced proliferative capacity of purified cells from the patient could in part be corrected by fetal bovine serum. The cells of the patient displayed a characteristic nucleoid sedimentation profile after ultraviolet irradiation indicating retarded DNA replication, which may be a common feature of various premature aging diseases. The absence of thermolability of cell proliferation and the presence of a high number of chromatid aberrations disclosed differences from classical WS.

摘要

对一名患有类似沃纳综合征(WS)的早衰患者的淋巴细胞增殖和核仁沉降进行了研究。该患者在9个月大时出现片状棕色色素沉着过度,这使其与经典的WS有所区别,并提示为一种类似WS的早衰疾病。通过光度记录细胞培养过程中的密度变化,我们检测了PHA刺激后7天内细胞增殖的过程,并将这些结果与两名正常对照者的结果进行了比较。该患者的培养细胞呈现出异常的增殖模式,其特征是持续生长而无初始减少阶段。患者纯化细胞显著降低的增殖能力部分可通过胎牛血清得到纠正。患者的细胞在紫外线照射后呈现出特征性的核仁沉降图谱,表明DNA复制延迟,这可能是各种早衰疾病的共同特征。细胞增殖缺乏热不稳定性以及存在大量染色单体畸变揭示了与经典WS的差异。

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