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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Jung, S. M. Articles by Moroi, M. Search for Related Content PubMed PubMed Citation Articles by Jung, S. M. Articles by Moroi, M. Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Thrombasthenia with an abnormal platelet membrane glycoprotein IIb of different molecular weight SM Jung, N Yoshida, N Aoki, K Tanoue, H Yamazaki and M Moroi Department of Biochemistry II, Jichi Medical School, Tochigi, Japan. We describe an individual with abnormal platelet glycoprotein (GP) IIb of different molecular weight (mol wt), a defect that distinguishes this patient from previously reported thrombasthenics. The patient, a 21-year-old female, has a mild bleeding tendency; her platelets lack adenosine diphosphate (ADP) aggregation and have severely suppressed collagen aggregation but a normal response to ristocetin. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis of her platelets indicates that they contain two types of GPIIb molecules: one with an abnormal mol wt (122 kd, unreduced; 128 kd, reduced) and one with a normal mol wt (128 kd, unreduced; 118 kd, reduced). Relative to the amount of GPIIb in normal platelets, her platelets contain approximately 35% abnormal GPIIb and 20% normal GPIIb. Fibrinogen binding assays on the patient's platelets indicated that they contained 25% of the normal amount of fibrinogen receptors. Crossed immunoelectrophoresis of the patient's platelets demonstrated the formation of a GPIIb/IIIa complex that was mainly composed of normal mol wt GPIIb and GPIIIa. The patient's father has decreased ADP aggregability, and his platelets also contained both abnormal and normal GPIIb (about 50% of the normal level and about 50% of the normal number of fibrinogen receptors); her mother has only normal GPIIb. These results indicate that the patient has heterozygous GPIIb molecules with an abnormality of GPIIb at the molecular level. Studies on this abnormal GPIIb should provide information about the function of GPIIb and the mechanism of its biosynthesis. Volume 71, Issue 4, pp. 915-922, 04/01/1988 Copyright © 1988 by The American Society of Hematology CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. Tadokoro, Y. Tomiyama, S. Honda, M. Arai, N. Yamamoto, M. Shiraga, S. Kosugi, Y. Kanakura, Y. Kurata, and Y. Matsuzawa A Gln747right-arrowPro Substitution in the alpha IIb Subunit Is Responsible for a Moderate alpha IIbbeta 3 Deficiency in Glanzmann Thrombasthenia Blood, October 15, 1998; 92(8): 2750 - 2758. [Abstract] [Full Text] [PDF]

	Copyright © 1988 by American Society of Hematology         Online ISSN: 1528-0020