再生障碍性贫血患儿骨髓造血干细胞端粒酶活性及相关基因表达的研究

王西阁; 周玉洁; 王丹凤; 王璇

doi:10.7499/j.issn.1008-8830.2013.01.007

PDF(1056 KB)

中国当代儿科杂志 ›› 2013, Vol. 15 ›› Issue (1) : 25-28. DOI: 10.7499/j.issn.1008-8830.2013.01.007

论著·临床研究

再生障碍性贫血患儿骨髓造血干细胞端粒酶活性及相关基因表达的研究

王西阁，周玉洁，王丹凤，王璇

作者信息 +

Expression of telomerase activity and its related genes in the marrow hemopoietic stem cells of children with aplastic anemia

WANG Xi-Ge, ZHOU Yu-Jie, WANG Dan-Feng, WANG Xuan

Author information +

文章历史 +

摘要

目的：探讨再生障碍性贫血（简称再障）患儿骨髓造血干细胞端粒酶活性及端粒酶逆转录酶（hTERT）基因和端粒酶RNA组分（hTERC）基因的表达变化和关系。方法：用重复序列扩增（TRAP）银染法和实时荧光定量RT-PCR法分别检测52例慢性再障患儿（CAA组）、13例急性再障患儿（SAA组）和21例骨髓造血正常儿童（对照组）端粒酶活性及hTERT和hTERC mRNA的表达。结果：CAA组、SAA组端粒酶活性和hTERT mRNA水平均高于对照组（P<0.01），且CAA组端粒酶活性和hTERT mRNA水平较SAA组升高（P<0.05）；hTERC mRNA在各组间表达差异无统计学意义（P=0.812）；hTERTmRNA的表达水平与端粒酶活性呈正相关（r＝0.660，P＜0.01）。结论：端粒酶活性的表达变化与儿童再障发病及发展过程有关，hTERT表达在端粒酶活性调节过程中起重要作用。

Abstract

OBJECTIVE: To explore the relationship of telomerase RNA component (hTERC) and the telomerase reverse transcriptase (hTERT) with telomerase activity in the marrow hemopoietic stem cells of children with aplastic anemia (AA). METHODS: Fifty-two children with chronic AA, 13 children with acute AA and 21 normal controls were enrolled in the study. Telomerase activity and the expression of mRNA of hTERT and hTERC were detected by Telomeric Repeat Amplification Protocol (TRAP) with silver staining and real-time Q-PCR respectively. RESULTS: Levels of telomerase activity in both the chronic and acute AA groups were higher than in the control group (P<0.01). The AA groups had significantly higher expression of hTERT mRNA than the control group (P<0.01). The chronic AA group had higher expression of hTERT mRNA and telomerase activity than the acute AA group (P<0.05). There was no significant difference in the expression of hTERC mRNA among the three groups (P=0.812). There was a significant correlation between the expression of hTERT mRNA and telomerase activity (r=0.660, P<0.01). CONCLUSIONS: Expression of telomerase activity may be involved in the pathophysiology and development of AA, and hTERT plays a crucial role in expression of telomerase activity.

导出引用

王西阁，周玉洁，王丹凤，王璇. 再生障碍性贫血患儿骨髓造血干细胞端粒酶活性及相关基因表达的研究[J]. 中国当代儿科杂志. 2013, 15(1): 25-28 https://doi.org/10.7499/j.issn.1008-8830.2013.01.007

WANG Xi-Ge, ZHOU Yu-Jie, WANG Dan-Feng, WANG Xuan. Expression of telomerase activity and its related genes in the marrow hemopoietic stem cells of children with aplastic anemia[J]. Chinese Journal of Contemporary Pediatrics. 2013, 15(1): 25-28 https://doi.org/10.7499/j.issn.1008-8830.2013.01.007

参考文献

［1］Ball SE, Gibson FM, Rizzo S,Tooze JA, Marsh JC, Gordon-Smith EC. Progressive telomere shortening in aplastic anemia［J］. Blood, 1998, 91(10): 35823592.

［2］中国小儿血液与肿瘤杂志编辑委员会. 小儿再生障碍性贫血诊疗建议［J］. 中国小儿血液与肿瘤杂志，2007，12（5）：236-240.

［3］申建宾，汤静燕，赵金彩，潘慈，陈静，薛惠良，等.再生障碍性贫血患者骨髓造血干细胞端粒酶活性异常及其意义的初步研究［J］.中华血液学杂志，2001，22（10）：543-545.

［4］Kamikozuru K, Fukunaga K, Hirota S, Hida N, Ohda Y, Yoshida K, et al. The expression profile of functional regulatory T cells,CD4+CD25high+/forkhead box protein P3+, in patients with ulcerative colitis during active and quiescent disease［J］. Clin Exp Immunol, 2009, 156(2): 320-327.

［5］王西阁，曹祎明，王晓格. CD4+CD25int /highCD127low调节性 T 细胞在再生障碍性贫血患儿中的检测及意义［J］.中国当代儿科杂志，2011，13(4): 292-293.

［7］张剑，付蓉，邵宗鸿. 获得性再生障碍性贫血患者遗传易感性研究进展［J］.中华血液学杂志，2010，31（11）：787-789.

［8］Abdellah BM, Haack-Sorensen M, Burns JS, Elsnab B, Jakob F, Hokland P, et al. Maintenance of differentiation potential of human bone marrow mesenchymal stem cells immoratalized by human telomerase reverse transeriptase gene despite extensive proliferation［J］.Biochem Biophys Res Commun, 2005, 326(3): 527-538.

［9］Mitchell JR, Wood E, Collins K. A telomerase component is defective in the human disease dyskeratosis congenital［J］. Nature, 1999, 402(6761): 551-555.

［10］Brummendorf TH, Maciejewski JP, Mak J, Young NS, Lansdorp PM. Telomere length in leukocyte subpopulations of patients with aplastie anemia［J］.Blood, 2001, 97(4): 895-900.

［11］Yamaguchi H, Calado RT, Ly H, Kajigaya S, Baerlocher JM, Chanock SJ, et al. Mutations in TERT, the gene for telomerase reverse transcriptase, in aplastic anemia［J］. N Engl J Med, 2005, 352(14): 1413-1424.

［12］Lansdorp PM. Telomeres, stem cells, and hematology［J］. Blood, 2008, 111(4): 1759-1766.

［13］Zhao YM, Li JY, Lan JP, Lai XY, Luo Y, Sun J, et al. Cell cycle dependent telomere regulation by telomerase in human bone marrow mesenchymal stem cells［J］. Biochemi Biophys Res Commu, 2008, 369(4): 1114-1119.

［14］Zimmermann S, Martens UM. Telomeres, senescence, and hematopoietic stem cells［J］. Cell Tissue Res, 2008, 331(1): 79-90.

［15］陈桂彬，邵宗鸿，贾海蓉，张益枝，郑以州，孙娟，等. 再生障碍性贫血患者骨髓造血干／祖细胞体外增殖分化特征的研究［J］.中华血液学杂志，1999，10：529-531.

［16］王西阁，王檬，刘松，王晓格，乔俊英，曹祎明，等. 环孢素对慢性再生障碍性贫血患儿外周血T 调节细胞及 Foxp3 表达的影响［J］. 中国当代儿科杂志，2011，13(12):936-939.