Source:http://linkedlifedata.com/resource/pubmed/id/15817705
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
1
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| pubmed:dateCreated |
2005-7-4
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| pubmed:abstractText |
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(-) 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(-) T cell compartments. In the GPI(-) T cells, severe defects in T cell receptor-dependent proliferation, interferon-gamma 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.
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| pubmed:language |
eng
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| pubmed:journal | |
| pubmed:citationSubset |
IM
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| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Antigens, CD,
http://linkedlifedata.com/resource/pubmed/chemical/Antigens, CD40,
http://linkedlifedata.com/resource/pubmed/chemical/CD40 Ligand,
http://linkedlifedata.com/resource/pubmed/chemical/CD48 antigen,
http://linkedlifedata.com/resource/pubmed/chemical/Intercellular Adhesion Molecule-1,
http://linkedlifedata.com/resource/pubmed/chemical/Interferon-gamma,
http://linkedlifedata.com/resource/pubmed/chemical/Membrane Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Receptors, Interleukin-2,
http://linkedlifedata.com/resource/pubmed/chemical/phosphatidylinositol glycan-class...
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| pubmed:status |
MEDLINE
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| pubmed:month |
Jul
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| pubmed:issn |
0741-5400
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| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:volume |
78
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| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
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| pubmed:pagination |
27-36
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| pubmed:dateRevised |
2008-11-21
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| pubmed:meshHeading |
pubmed-meshheading:15817705-Adult,
pubmed-meshheading:15817705-Antigens, CD,
pubmed-meshheading:15817705-Antigens, CD40,
pubmed-meshheading:15817705-CD4-Positive T-Lymphocytes,
pubmed-meshheading:15817705-CD40 Ligand,
pubmed-meshheading:15817705-Cell Proliferation,
pubmed-meshheading:15817705-Female,
pubmed-meshheading:15817705-Hemoglobinuria, Paroxysmal,
pubmed-meshheading:15817705-Humans,
pubmed-meshheading:15817705-Intercellular Adhesion Molecule-1,
pubmed-meshheading:15817705-Interferon-gamma,
pubmed-meshheading:15817705-Lymphopenia,
pubmed-meshheading:15817705-Male,
pubmed-meshheading:15817705-Membrane Proteins,
pubmed-meshheading:15817705-Mutation,
pubmed-meshheading:15817705-Receptors, Interleukin-2,
pubmed-meshheading:15817705-Signal Transduction,
pubmed-meshheading:15817705-T-Lymphocytes
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| pubmed:year |
2005
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| pubmed:articleTitle |
T cells from paroxysmal nocturnal haemoglobinuria (PNH) patients show an altered CD40-dependent pathway.
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| pubmed:affiliation |
Cattedra de Immunologia, Dipartimento de Biologia e Pathologia Cellulare e Molecolare, Naples, Italy.
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| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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