{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T12:31:19Z","timestamp":1788870679036,"version":"build-2803163510"},"reference-count":57,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2005,4,7]],"date-time":"2005-04-07T00:00:00Z","timestamp":1112832000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Ricerca Scientifica","award":["2002"],"award-info":[{"award-number":["2002"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2005,4,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Paroxysmal nocturnal haemoglobinuria (PNH) is a haematopoiesis disorder characterized by the expansion of a stem cell bearing a somatic mutation in the phosphatidylinositol glycan-A (PIG-A) gene, which is involved in the biosynthesis of the glycosylphosphatidylinositol (GPI) anchor. A number of data suggest the inability of the PIG-A mutation to account alone for the clonal dominance of the GPI-defective clone and for the development of PNH. In this context, additional immune-mediated mechanisms have been hypothesized. We focused on the analysis of T lymphocytes in three PNH patients bearing a mixed GPI+ and GPI\u2013 T cell population and showing a marked cytopenia. To analyze the biological mechanisms underlying the control of T cell homeostasis in PNH, we addressed the study of CD40-dependent pathways, suggested to be of crucial relevance for the control of autoreactive T cell clones. Our data revealed significant, functional alterations in GPI+ and GPI\u2013 T cell compartments. In the GPI\u2013 T cells, severe defects in T cell receptor-dependent proliferation, interferon-\u03b3 production, CD25, CD54, and human leukocyte antigen-DR surface expression were observed. By contrast, GPI+ T lymphocytes showed a significant increase of all these parameters, and the analysis of CD40-dependent pathways revealed a functional persistence of CD154 expression on the CD48+CD4+ lymphocytes. The alterations of the GPI+ T cell subset could be involved in the biological mechanisms underlying PNH pathogenesis.<\/jats:p>","DOI":"10.1189\/jlb.0105026","type":"journal-article","created":{"date-parts":[[2005,4,7]],"date-time":"2005-04-07T20:33:52Z","timestamp":1112906032000},"page":"27-36","source":"Crossref","is-referenced-by-count":14,"title":["T cells from paroxysmal nocturnal haemoglobinuria (PNH) patients show an altered CD40-dependent pathway"],"prefix":"10.1093","volume":"78","author":[{"given":"Giuseppe","family":"Terrazzano","sequence":"first","affiliation":[{"name":"Cattedra di Immunologia, Dipartimento di Biologia e Patologia Cellulare e Molecolare, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michela","family":"Sica","sequence":"additional","affiliation":[{"name":"Cattedra di Immunologia, Dipartimento di Biologia e Patologia Cellulare e Molecolare, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Becchimanzi","sequence":"additional","affiliation":[{"name":"Cattedra di Ematologia, Dipartimento di Medicina Clinica e Sperimentale, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Costantini","sequence":"additional","affiliation":[{"name":"Cattedra di Ematologia, Dipartimento di Medicina Clinica e Sperimentale, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Rotoli","sequence":"additional","affiliation":[{"name":"Cattedra di Ematologia, Dipartimento di Medicina Clinica e Sperimentale, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Serafino","family":"Zappacosta","sequence":"additional","affiliation":[{"name":"Cattedra di Immunologia, Dipartimento di Biologia e Patologia Cellulare e Molecolare, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fiorella","family":"Alfinito","sequence":"additional","affiliation":[{"name":"Cattedra di Ematologia, Dipartimento di Medicina Clinica e Sperimentale, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppina","family":"Ruggiero","sequence":"additional","affiliation":[{"name":"Cattedra di Immunologia, Dipartimento di Biologia e Patologia Cellulare e Molecolare, Universit\u00e0 di Napoli \u201cFederico II\u201d , Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2005,4,7]]},"reference":[{"key":"2023033022491374200_","first-page":"201","article-title":"Paroxysmal nocturnal hemoglobinuria","volume":"26","author":"Rotoli","year":"1989","journal-title":"Semin. 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