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腺苷脱氨酶缺乏的淋巴细胞中嘌呤核苷酸增加。

Increased purine nucleotides in adenosine deaminase-deficient lymphocytes.

作者信息

Schmalstieg F C, Nelson J A, Mills G C, Monahan T M, Goldman A S, Goldblum R M

出版信息

J Pediatr. 1977 Jul;91(1):48-51. doi: 10.1016/s0022-3476(77)80442-3.

DOI:10.1016/s0022-3476(77)80442-3
PMID:195028
Abstract

It was found that ATP and cyclic AMP were greatly increased in human blood lymphocytes which were deficient in ADA. Certain other purine and pyrimidine nucleotides were elevated but to a lesser degree. Energy production in these cells may be inhibited by the increase in nucleotides since the ATP:ADP ratio was significantly below normal. Thus it appears that the immunologic deficiency in human ADA deficiency is related to increased nucleotide concentrations in the lymphocytes.

摘要

研究发现,在缺乏腺苷脱氨酶(ADA)的人血淋巴细胞中,三磷酸腺苷(ATP)和环磷酸腺苷(cAMP)大幅增加。某些其他嘌呤和嘧啶核苷酸也有所升高,但程度较轻。由于ATP与二磷酸腺苷(ADP)的比率显著低于正常水平,这些细胞中的能量产生可能受到核苷酸增加的抑制。因此,人类ADA缺乏症中的免疫缺陷似乎与淋巴细胞中核苷酸浓度的增加有关。

相似文献

1
Increased purine nucleotides in adenosine deaminase-deficient lymphocytes.腺苷脱氨酶缺乏的淋巴细胞中嘌呤核苷酸增加。
J Pediatr. 1977 Jul;91(1):48-51. doi: 10.1016/s0022-3476(77)80442-3.
2
Purine metabolism in adenosine deaminase deficiency.腺苷脱氨酶缺乏症中的嘌呤代谢
Proc Natl Acad Sci U S A. 1976 Aug;73(8):2867-71. doi: 10.1073/pnas.73.8.2867.
3
Purine dysfunction in cells from patients with adenosine deaminase deficiency.腺苷脱氨酶缺乏症患者细胞中的嘌呤功能障碍。
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Purine metabolism in adenosine deaminase deficiency.腺苷脱氨酶缺乏症中的嘌呤代谢
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Purine nucleoside metabolism in the erythrocytes of patients with adenosine deaminase deficiency and severe combined immunodeficiency.腺苷脱氨酶缺乏和严重联合免疫缺陷患者红细胞中的嘌呤核苷代谢
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Adenine and adenosine metabolism in lymphocytes deficient in adenosine deaminase (ADA) activity.腺苷脱氨酶(ADA)活性缺乏的淋巴细胞中的腺嘌呤和腺苷代谢
Adv Exp Med Biol. 1977;76A:456-62. doi: 10.1007/978-1-4613-4223-6_57.
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Adenosine deaminase deficiency and severe combined immunodeficiency disease.腺苷脱氨酶缺乏症与重症联合免疫缺陷病
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[Immune insufficiency in enzyme defects of purine metabolism].[嘌呤代谢酶缺陷中的免疫功能不全]
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Immunological and metabolic reconstitution following successful bone marrow transplantation from a HLA-identical sibling in an infant with adenosine deaminase deficiency and severe combined immunodeficiency: partial restoration of purine metabolism.一名患有腺苷脱氨酶缺乏症和严重联合免疫缺陷的婴儿接受来自 HLA 相同同胞的成功骨髓移植后的免疫和代谢重建:嘌呤代谢的部分恢复
Adv Exp Med Biol. 1989;253A:543-7. doi: 10.1007/978-1-4684-5673-8_88.

引用本文的文献

1
Biochemical and functional abnormalities in lymphocytes from an adenosine deaminase-deficient patient during enzyme replacement therapy.腺苷脱氨酶缺乏症患者在酶替代治疗期间淋巴细胞的生化及功能异常
J Clin Invest. 1981 Aug;68(2):413-21. doi: 10.1172/jci110270.
2
Bone marrow transplantation only partially restores purine metabolites to normal in adenosine deaminase-deficient patients.对于腺苷脱氨酶缺乏症患者,骨髓移植只能使嘌呤代谢产物部分恢复正常。
J Clin Invest. 1981 Dec;68(6):1387-93. doi: 10.1172/jci110389.
3
Effect of red cell transfusions, thymic hormone and deoxycytidine in severe combined immunodeficiency due to adenosine deaminase deficiency.
红细胞输注、胸腺激素和脱氧胞苷对腺苷脱氨酶缺乏所致重症联合免疫缺陷的影响。
Clin Exp Immunol. 1982 Nov;50(2):303-10.
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Canine cyclic hematopoiesis is associated with abnormal purine and pyrimidine metabolism.犬类周期性造血与嘌呤和嘧啶代谢异常有关。
J Clin Invest. 1983 May;71(5):1348-55. doi: 10.1172/jci110887.
5
Adenosine deaminase in human epidermis from healthy and psoriatic subjects.来自健康和银屑病患者的人表皮中的腺苷脱氨酶
Arch Dermatol Res. 1983;275(5):310-4. doi: 10.1007/BF00417203.
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J Clin Invest. 1980 Sep;66(3):543-50. doi: 10.1172/JCI109886.
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J Clin Immunol. 1985 May;5(3):180-6. doi: 10.1007/BF00915509.
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