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May-Hegglin异常患者血小板中糖蛋白异常的证据。

Evidence for glycoprotein abnormality in platelets from patients with May-Hegglin anomaly.

作者信息

Ricci G, Manservigi R, Albonici L, Zavagli G, Cassai E

出版信息

Thromb Haemost. 1985 Dec 17;54(4):862-5.

PMID:4089820
Abstract

In the present research protein analysis on SDS-polyacrylamide gel electrophoresis (PAGE) has been used to study the glycoprotein pattern of the blood platelets of four members from a family affected by May-Hegglin anomaly. In order to characterize the glycoprotein fractions, lactoperoxidase-iodination and immunoprecipitation procedures were used. N,N'diallyltartardiamide (DATD) cross-linked gel electrophoresis was shown to improve the glycoprotein pattern resolution, although with the lactoperoxidase-iodination the glycoprotein characterization of May-Hegglin platelets overlapped the normal. On the other hand with the immunoprecipitation the specific antiserum precipitated all the five major fractions of normal membrane glycoproteins, but it was shown to react quite poorly with the component V of the May-Hegglin glycoprotein pattern.

摘要

在本研究中,利用十二烷基硫酸钠-聚丙烯酰胺凝胶电泳(PAGE)进行蛋白质分析,以研究一个受May-Hegglin异常影响的家族中四名成员血小板的糖蛋白模式。为了表征糖蛋白组分,采用了乳过氧化物酶碘化和免疫沉淀程序。结果表明,N,N'-二烯丙基酒石酰胺(DATD)交联凝胶电泳可提高糖蛋白模式的分辨率,不过在乳过氧化物酶碘化过程中,May-Hegglin血小板的糖蛋白特征与正常情况重叠。另一方面,通过免疫沉淀,特异性抗血清沉淀出了正常膜糖蛋白的所有五个主要组分,但结果显示其与May-Hegglin糖蛋白模式的组分V反应很差。

相似文献

1
Evidence for glycoprotein abnormality in platelets from patients with May-Hegglin anomaly.May-Hegglin异常患者血小板中糖蛋白异常的证据。
Thromb Haemost. 1985 Dec 17;54(4):862-5.
2
Immunochemical evidence for protein abnormalities in platelets from patients with Glanzmann's thrombasthenia and Bernard-Soulier syndrome.血小板无力症和巨大血小板综合征患者血小板中蛋白质异常的免疫化学证据。
J Clin Invest. 1980 Mar;65(3):722-31. doi: 10.1172/JCI109719.
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The use of functional and quantitative assays to study glycoprotein Ib in platelets stored under various in vitro conditions.
Thromb Haemost. 1984 Dec 29;52(3):271-5.
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Pregnancy complicated by rhesus sensitization and the May-Hegglin anomaly.妊娠合并恒河猴致敏和May-Hegglin异常。
Obstet Gynecol. 1985 Mar;65(3 Suppl):7S-10S.
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An altered platelet granule glycoprotein in patients with essential thrombocythemia.原发性血小板增多症患者血小板颗粒糖蛋白的改变
J Clin Invest. 1984 Feb;73(2):291-7. doi: 10.1172/JCI111213.
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[Hematological and genetic investigations of a family affected with May-Hegglin anomaly].[一个患有May-Hegglin异常的家族的血液学和遗传学研究]
Arch Genet (Zur). 1975;48(1):47-9.
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Detection of carriers in Glanzmann's thrombasthenia.Glanzmann血小板无力症携带者的检测。
Thromb Haemost. 1983 Jun 28;49(3):182-6.
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Studies on subcellular fractions of human platelets by the lactoperoxidase-iodination technique.
Biochim Biophys Acta. 1976 Nov 11;455(1):214-25. doi: 10.1016/0005-2736(76)90165-6.
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Molecular defects of platelets in Bernard-Soulier syndrome.伯纳德-索利尔综合征中血小板的分子缺陷
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Thromb Haemost. 1980 Feb 29;42(5):1490-502.

引用本文的文献

1
Hereditary types of thrombocytopenia with giant platelets and inclusion bodies in the leukocytes.
Blut. 1990 Feb;60(2):53-60. doi: 10.1007/BF01720508.
2
May-Hegglin anomaly: a rare cause of thrombocytopenia.
Eur J Pediatr. 1992 Sep;151(9):668-71. doi: 10.1007/BF01957570.