K A Siminovitch, W L Greer, B Axelsson, L A Rubin, A Novogrodsky, M Peacocke
{"title":"Wiskott-Aldrich综合征中cd43介导的T细胞活化的选择性损伤。","authors":"K A Siminovitch, W L Greer, B Axelsson, L A Rubin, A Novogrodsky, M Peacocke","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>The Wiskott-Aldrich syndrome (WAS) is associated with defective glycosylation and altered membrane expression of the leukocyte sialoglycoprotein CD43. To investigate whether such modifications of CD43 are relevant to T cell dysfunction in WAS, we have analyzed peripheral blood mononuclear cells from WAS patients for proliferative responses to both CD43-interacting and other T cell mitogenic stimuli. While patient lymphocytes proliferated in response to phytohaemagglutinin, concanavalin A, interleukin-2 and neuraminidase/galactose oxidase, no responses were elicited upon attempted triggering of the CD43 signalling pathway using periodate or anti-CD43 antibody. Cells from four of five patients with clinical profiles resembling, but not identical, to that of classic WAS also failed to respond to periodate or anti-CD43 antibody stimulation. These results indicate that the aberrant expression of CD43 on WAS lymphocytes is associated with selective impairment of CD43-induced T cell proliferation and that detection of this defect may be useful in the diagnosis of WAS and its variant forms.</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Selective impairment of CD43-mediated T cell activation in the Wiskott-Aldrich syndrome.\",\"authors\":\"K A Siminovitch, W L Greer, B Axelsson, L A Rubin, A Novogrodsky, M Peacocke\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>The Wiskott-Aldrich syndrome (WAS) is associated with defective glycosylation and altered membrane expression of the leukocyte sialoglycoprotein CD43. To investigate whether such modifications of CD43 are relevant to T cell dysfunction in WAS, we have analyzed peripheral blood mononuclear cells from WAS patients for proliferative responses to both CD43-interacting and other T cell mitogenic stimuli. While patient lymphocytes proliferated in response to phytohaemagglutinin, concanavalin A, interleukin-2 and neuraminidase/galactose oxidase, no responses were elicited upon attempted triggering of the CD43 signalling pathway using periodate or anti-CD43 antibody. Cells from four of five patients with clinical profiles resembling, but not identical, to that of classic WAS also failed to respond to periodate or anti-CD43 antibody stimulation. These results indicate that the aberrant expression of CD43 on WAS lymphocytes is associated with selective impairment of CD43-induced T cell proliferation and that detection of this defect may be useful in the diagnosis of WAS and its variant forms.</p>\",\"PeriodicalId\":79340,\"journal\":{\"name\":\"Immunodeficiency\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1993-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Immunodeficiency\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Immunodeficiency","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Selective impairment of CD43-mediated T cell activation in the Wiskott-Aldrich syndrome.
The Wiskott-Aldrich syndrome (WAS) is associated with defective glycosylation and altered membrane expression of the leukocyte sialoglycoprotein CD43. To investigate whether such modifications of CD43 are relevant to T cell dysfunction in WAS, we have analyzed peripheral blood mononuclear cells from WAS patients for proliferative responses to both CD43-interacting and other T cell mitogenic stimuli. While patient lymphocytes proliferated in response to phytohaemagglutinin, concanavalin A, interleukin-2 and neuraminidase/galactose oxidase, no responses were elicited upon attempted triggering of the CD43 signalling pathway using periodate or anti-CD43 antibody. Cells from four of five patients with clinical profiles resembling, but not identical, to that of classic WAS also failed to respond to periodate or anti-CD43 antibody stimulation. These results indicate that the aberrant expression of CD43 on WAS lymphocytes is associated with selective impairment of CD43-induced T cell proliferation and that detection of this defect may be useful in the diagnosis of WAS and its variant forms.