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活性T细胞和抑制性T细胞:先天性角化不良患者及其一级亲属中的减少情况。

Active and suppressor T cells: diminution in a patient with dyskeratosis congenita and in first-degree relatives.

作者信息

Fudenberg H H, Goust J M, Vesole D H, Salinas C F

出版信息

Gerontology. 1979;25(4):231-7. doi: 10.1159/000212345.

DOI:10.1159/000212345
PMID:313356
Abstract

Active, total and nonspecific suppressor T cells were studied in a 15-year-old black male with dyskeratosis congenita syndrome, a precancerous mucosal disease, and in 7 siblings and several other relatives in three generations. The propositus and 1 elder sister, products of a second-cousin marriage, died with dyskeratosis congenita. The mother had dermatomyositis, and the maternal grandmother and her sister reportedly had rheumatoid arthritis. Studies of available siblings, father, and grandparents revealed a high incidence of deficiency in number of active and/or suppressor T cells, sometimes severe enough to result in a decrease in total T cells. The patient had many stigmata of precocious aging, as did the sibling who died with the same syndrome. The laboratory data suggest that a defect in cell-mediated immunity, involving mainly or exclusively suppressor T cells, is associated with, and is presumably the cause of, precocious aging; perhaps an abiotrophy in this cell subpopulation results in physiologic aging.

摘要

对一名患有先天性角化不良综合征(一种癌前黏膜疾病)的15岁黑人男性以及其三代内的7名兄弟姐妹和其他几名亲属进行了活性、总抑制性和非特异性抑制性T细胞研究。先证者和一名表姐婚姻所生的姐姐死于先天性角化不良。母亲患有皮肌炎,据报道外祖母及其妹妹患有类风湿性关节炎。对在世的兄弟姐妹、父亲和祖父母的研究显示,活性和/或抑制性T细胞数量不足的发生率很高,有时严重到足以导致总T细胞数量减少。该患者有许多早衰的体征,死于相同综合征的兄弟姐妹也是如此。实验室数据表明,主要或仅涉及抑制性T细胞的细胞介导免疫缺陷与早衰相关,并且可能是早衰的原因;也许这个细胞亚群的营养缺陷导致了生理衰老。

相似文献

1
Active and suppressor T cells: diminution in a patient with dyskeratosis congenita and in first-degree relatives.活性T细胞和抑制性T细胞:先天性角化不良患者及其一级亲属中的减少情况。
Gerontology. 1979;25(4):231-7. doi: 10.1159/000212345.
2
Bone marrow failure in dyskeratosis congenita.先天性角化不良中的骨髓衰竭。
Scand J Haematol. 1984 May;32(5):496-500. doi: 10.1111/j.1600-0609.1984.tb02191.x.
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Abnormality of platelet size and T-lymphocyte proliferation in an autosomal recessive form of dyskeratosis congenita.常染色体隐性遗传性先天性角化不良中血小板大小及T淋巴细胞增殖异常
Eur J Haematol. 1987 Oct;39(4):306-10. doi: 10.1111/j.1600-0609.1987.tb00774.x.
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Zinsser-Cole-Engman syndrome (dyskeratosis congenita) with cataract--a rare association.津瑟-科尔-恩格曼综合征(先天性角化不良)合并白内障——一种罕见的关联。
Jpn J Ophthalmol. 1986;30(2):192-6.
5
X-linked dyskeratosis congenita with pancytopenia.伴有全血细胞减少的X连锁先天性角化不良
Arch Dermatol. 1978 Nov;114(11):1667-71.
6
T cell immunodeficiency in dyskeratosis congenita.先天性角化不良中的T细胞免疫缺陷。
Arch Dis Child. 1992 Apr;67(4):524-6. doi: 10.1136/adc.67.4.524.
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Dyskeratosis congenita: an autosomal dominant disorder.
J Am Acad Dermatol. 1982 Jun;6(6):1034-9. doi: 10.1016/s0190-9622(82)80100-x.
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Psoralen-DNA cross-linking photoadducts in dyskeratosis congenita: delay in excision and promotion of sister chromatid exchange.
J Invest Dermatol. 1979 Jul;73(1):97-101. doi: 10.1111/1523-1747.ep12532783.
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T cells, precocious aging, and familial neoplasia.
Gerontology. 1978;24(4):266-75. doi: 10.1159/000212259.
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Progressive immune failure in dyskeratosis congenita. Report of an adult in whom Pneumocystis carinii and fatal disseminated candidiasis developed.先天性角化不良中的进行性免疫衰竭。一例发生卡氏肺孢子虫感染和致命播散性念珠菌病的成人病例报告。
Arch Intern Med. 1984 Feb;144(2):397-9.

引用本文的文献

1
Telomerase insufficiency in rheumatoid arthritis.类风湿关节炎中的端粒酶不足
Proc Natl Acad Sci U S A. 2009 Mar 17;106(11):4360-5. doi: 10.1073/pnas.0811332106. Epub 2009 Mar 2.
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Enhanced G2 chromatid radiosensitivity in dyskeratosis congenita fibroblasts.先天性角化不良成纤维细胞中增强的G2染色单体放射敏感性。
Am J Hum Genet. 1990 Feb;46(2):350-7.
3
Dyskeratosis congenita (Zinsser-Cole-Engman syndrome). An autopsy case presenting with rectal carcinoma, non-cirrhotic portal hypertension, and Pneumocystis carinii pneumonia.
先天性角化不良(津瑟-科尔-恩格曼综合征)。一例尸检病例,伴有直肠癌、非肝硬化性门静脉高压症和卡氏肺孢子虫肺炎。
Virchows Arch A Pathol Anat Histopathol. 1990;417(3):247-53. doi: 10.1007/BF01600141.