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Immunodeficiency最新文献

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Bone marrow transplantation in severe combined immunodeficiency: potential and current limitations. 骨髓移植治疗严重联合免疫缺陷:潜在的和目前的局限性。
Immunodeficiency Pub Date : 1993-01-01
W Friedrich, C Knobloch, J Greher, W Hartmann, H H Peter, S F Goldmann, E Kleihauer
{"title":"Bone marrow transplantation in severe combined immunodeficiency: potential and current limitations.","authors":"W Friedrich, C Knobloch, J Greher, W Hartmann, H H Peter, S F Goldmann, E Kleihauer","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effector functions of IgG subclass antibodies. IgG亚类抗体的效应功能。
Immunodeficiency Pub Date : 1993-01-01
R G Bredius, J G Van de Winkel, R S Weening, T A Out
{"title":"Effector functions of IgG subclass antibodies.","authors":"R G Bredius, J G Van de Winkel, R S Weening, T A Out","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Control of human B cell tumor growth in SCID mice by monoclonal anti-B cell antibodies. 单克隆抗B细胞抗体对SCID小鼠人B细胞肿瘤生长的控制。
Immunodeficiency Pub Date : 1993-01-01
A Durandy, N Brousse, F Rozenberg, G De Saint Basile, A M Fischer, A Fischer
{"title":"Control of human B cell tumor growth in SCID mice by monoclonal anti-B cell antibodies.","authors":"A Durandy, N Brousse, F Rozenberg, G De Saint Basile, A M Fischer, A Fischer","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158270","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Selective impairment of CD43-mediated T cell activation in the Wiskott-Aldrich syndrome. Wiskott-Aldrich综合征中cd43介导的T细胞活化的选择性损伤。
Immunodeficiency Pub Date : 1993-01-01
K A Siminovitch, W L Greer, B Axelsson, L A Rubin, A Novogrodsky, M Peacocke
{"title":"Selective impairment of CD43-mediated T cell activation in the Wiskott-Aldrich syndrome.","authors":"K A Siminovitch,&nbsp;W L Greer,&nbsp;B Axelsson,&nbsp;L A Rubin,&nbsp;A Novogrodsky,&nbsp;M Peacocke","doi":"","DOIUrl":"","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":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The influence of gene duplications in the human immunoglobulin heavy chain constant locus on serum levels of immunoglobulin. 人免疫球蛋白重链常数位点基因重复对血清免疫球蛋白水平的影响。
Immunodeficiency Pub Date : 1993-01-01
H Rabbani, A Bottaro, A O Carbonara, A Brusco, U Cariota, P G Olsson, C I Smith, L Hammarström
{"title":"The influence of gene duplications in the human immunoglobulin heavy chain constant locus on serum levels of immunoglobulin.","authors":"H Rabbani,&nbsp;A Bottaro,&nbsp;A O Carbonara,&nbsp;A Brusco,&nbsp;U Cariota,&nbsp;P G Olsson,&nbsp;C I Smith,&nbsp;L Hammarström","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Biosynthesis of Tcr-alpha, beta and Tcr-gamma, delta/CD3 complexes. tcr - α， β和tcr - γ， δ /CD3复合物的生物合成。
Immunodeficiency Pub Date : 1993-01-01
S Bauguil-Caspar, J Arnaud, J Kuhlmann, C Geisler, B Rubin
{"title":"Biosynthesis of Tcr-alpha, beta and Tcr-gamma, delta/CD3 complexes.","authors":"S Bauguil-Caspar,&nbsp;J Arnaud,&nbsp;J Kuhlmann,&nbsp;C Geisler,&nbsp;B Rubin","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Jurkat J76 clone, LYON L12.37 clone and L12.37 cells transfected with J76-alpha cDNA or J76 Tcr-alpha mutated cDNA (J79) were analysed for membrane expression of Tcr/CD3 complex using WT31 mAb (Tcr-alpha, beta) or Tcr-delta 1 mAb (Tcr-gamma, delta): LYON cells express V beta 9 bearing Tcr-beta chains. J76 Tcr-alpha cDNA transfected LYON cells have intracellular Tcr-gamma, delta chains and J79 Tcr-alpha cDNA transfected LYON cells have intracellular Tcr-alpha (M), beta chains.</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158476","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Characterization of anergy to the superantigen Staphylococcus enterotoxin B. 对超抗原葡萄球菌肠毒素B的能量表征。
Immunodeficiency Pub Date : 1993-01-01
G Dannecker, H Schultz, S Mecheri, G Sappler, K Clarke, D Niethammer, M K Hoffmann
{"title":"Characterization of anergy to the superantigen Staphylococcus enterotoxin B.","authors":"G Dannecker,&nbsp;H Schultz,&nbsp;S Mecheri,&nbsp;G Sappler,&nbsp;K Clarke,&nbsp;D Niethammer,&nbsp;M K Hoffmann","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>In vivo administration of superantigens leads to activation and subsequent depletion or anergy of T cells expressing defined V beta-T cell receptors (TCR). Superantigens have therefore become intensively studied tools for examining parameters of immunoregulation and they may represent model antigens for pathogenic agents. An HIV-encoded superantigen has for example been implicated in the dramatic loss of helper T cells in AIDS. We investigated the response of V beta 8+ T cells in mice after primary and secondary exposure to the superantigen Staphylococcus Enterotoxin B (SEB).</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19159039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Structure of the NADPH-oxidase: membrane components. nadph氧化酶的结构:膜组分。
Immunodeficiency Pub Date : 1993-01-01
A W Segal
{"title":"Structure of the NADPH-oxidase: membrane components.","authors":"A W Segal","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19159048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Bone marrow gene therapy for adenosine deaminase deficiency. 骨髓基因治疗腺苷脱氨酶缺乏症。
Immunodeficiency Pub Date : 1993-01-01
L C Kaptein, M P Einerhand, E Braakman, D Valerio, V W van Beusechem
{"title":"Bone marrow gene therapy for adenosine deaminase deficiency.","authors":"L C Kaptein,&nbsp;M P Einerhand,&nbsp;E Braakman,&nbsp;D Valerio,&nbsp;V W van Beusechem","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Deficiency of adenosine deaminase (ADA) results in severe combined immunodeficiency disease (SCID). The cause for this is believed to be the accumulation of one of the substrates for ADA, 2'-deoxyadenosine to which especially T cells are hypersensitive. This disease can be treated successfully with bone marrow transplantation if a suitable donor is available. Alternatively, the human ADA gene could be introduced into the autologous bone marrow. We have generated a retroviral vector containing the human ADA gene. With this vector we were able to restore human ADA-activity in ADA-SCID T cells to normal levels resulting in a sensitivity to 2'-deoxyadenosine that is also found for T cells from a healthy donor. In murine studies we have shown that our retrovirus can infect pluripotent hemopoietic stem cells resulting in long-term (> 6 months) expression of human ADA in the hemopoietic system of transplanted animals. These results were confirmed in rhesus monkeys where we were able to detect the provirus in both peripheral blood mononuclear cells and granulocytes for as long as the animals were analyzed, i.e. up to more than 1 year post bone marrow transplantation. On the basis of these results we have proposed a clinical protocol for the treatment of ADA-SCID patients with bone marrow gene therapy.</p>","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"18907037","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical and immunological follow-up in children with hyper-IgD syndrome. 儿童高igd综合征的临床和免疫学随访。
Immunodeficiency Pub Date : 1993-01-01
A Haraldsson, C M Weemaes, A W de Boer, J A Bakkeren
{"title":"Clinical and immunological follow-up in children with hyper-IgD syndrome.","authors":"A Haraldsson,&nbsp;C M Weemaes,&nbsp;A W de Boer,&nbsp;J A Bakkeren","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":79340,"journal":{"name":"Immunodeficiency","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"1993-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"19158267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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