Identificador persistente para citar o vincular este elemento: http://hdl.handle.net/10553/48651
Campo DC Valoridioma
dc.contributor.authorArnaiz-Villena, A.en_US
dc.contributor.authorTimon, M.en_US
dc.contributor.authorRodriguez-Gallego, C.en_US
dc.contributor.authorIglesias-Casarrubios, P.en_US
dc.contributor.authorPacheco, A.en_US
dc.contributor.authorRegueiro, J. R.en_US
dc.date.accessioned2018-11-23T23:44:49Z-
dc.date.available2018-11-23T23:44:49Z-
dc.date.issued1993en_US
dc.identifier.issn1067-795Xen_US
dc.identifier.urihttp://hdl.handle.net/10553/48651-
dc.description.abstractA selective CD3 gamma defect, involving point mutations in both the paternal and the maternal genes, has been analyzed. The CD3 gamma defect affected two brothers of a four sibs family; one of them died at the age of 3 of a viral pneumonia with concomitant autoimmune features (Haemolytic anaemia and gut epithelial cell autoantibodies), whereas the other is still alive at the age of 10 with relatively mild infection episodes. In this work, the effects of the absence of the CD3 gamma chain in the structure and signal transduction of the T-cell receptor (TCR)/CD3 complex and in the selection and function of T lymphocytes were studied. The absence of CD3 gamma did not prevent the expression of certain amounts of TCR/CD3 complexes on the surface of T lymphocytes. This suggests the existence of at least two TCR/CD3 isoforms in T cells, either with or without CD3 gamma. A persistent low proportion of CD8+ T cells, not functional in vitro (they were unable to proliferate) and probably in vivo (associated to a lethal viral pneumonia), was observed. In contrast, the proportion of CD4+ T cells was not altered, and they were functional both in vitro (they showed a normal proliferation and a low, but detectable, increase of cytosolic Ca2+ in response to anti-TCR/CD3 stimuli, although the production of IL-2 was impaired) and in vitro (they normally helped B cells). These results show that the absence of CD3 gamma affects the selection and function of cytotoxic, but apparently not helper, T lymphocytes, although the possibility that the CD4+ T cells represent a specific subpopulation can not be ruled out. They also suggest that the interactions of the TCR/CD3 complex with its co-receptors during thymic selection and antigen recognition in the periphery may be asymmetrical, with CD8 interacting through CD3 gamma and, probably, CD4 through the homologous CD3 delta.en_US
dc.languageengen_US
dc.relation.ispartofImmunodeficiencyen_US
dc.sourceImmunodeficiency[ISSN 1067-795X],v. 4, p. 121-129en_US
dc.subject32 Ciencias médicasen_US
dc.subject310903 Inmunologíaen_US
dc.subject.otherCD4-Positive T-Lymphocytesen_US
dc.subject.otherCD8 Antigensen_US
dc.subject.otherImmunologic Deficiency Syndromesen_US
dc.titleT lymphocyte signalling defects and immunodeficiency due to the lack of CD3γen_US
dc.typeinfo:eu-repo/semantics/conferenceObjecten_US
dc.typeConferenceObjecten_US
dc.identifier.scopus0027770892-
dc.contributor.authorscopusid7102651426-
dc.contributor.authorscopusid7004415077-
dc.contributor.authorscopusid6602114379-
dc.contributor.authorscopusid6603364789-
dc.contributor.authorscopusid57197346439-
dc.contributor.authorscopusid7005510950-
dc.description.lastpage129en_US
dc.description.firstpage121en_US
dc.relation.volume4en_US
dc.investigacionCiencias de la Saluden_US
dc.type2Actas de congresosen_US
dc.description.numberofpages9en_US
dc.utils.revisionen_US
dc.identifier.ulpgcen_US
dc.contributor.buulpgcBU-MEDen_US
item.grantfulltextnone-
item.fulltextSin texto completo-
crisitem.author.deptGIR IUIBS: Farmacología Molecular y Traslacional-
crisitem.author.deptIU de Investigaciones Biomédicas y Sanitarias-
crisitem.author.deptDepartamento de Ciencias Médicas y Quirúrgicas-
crisitem.author.orcid0000-0002-4344-8644-
crisitem.author.parentorgIU de Investigaciones Biomédicas y Sanitarias-
crisitem.author.fullNameRodríguez Gallego, José Carlos-
Colección:Actas de congresos
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