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Glycosylphosphatidylinositol-specific, CD1d-restricted T cells in paroxysmal nocturnal hemoglobinuria.

Abstract:

The mechanism of bone marrow failure (BMF) in paroxysmal nocturnal hemoglobinuria (PNH) is not yet known. Because in PNH the biosynthesis of the glycolipid molecule glycosylphosphatidylinositol (GPI) is disrupted in hematopoietic stem and progenitor cells by a somatic mutation in the PIG-A gene, BMF might result from an autoimmune attack, whereby T cells target GPI in normal cells, whereas PIG-A mutant GPI-negative cells are spared. In a deliberate test of this hypothesis, we have demonstrate...

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Publication status:
Published
Peer review status:
Peer reviewed

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Associazione Italiana per la Ricerca sul Cancro More from this funder
National Institute for Health Research More from this funder
Regione Toscana More from this funder
Italian Ministry of Education, University and Research More from this funder
Imperial Healthcare Charity More from this funder
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Publisher:
American Society of Hematology Publisher's website
Journal:
Blood Journal website
Volume:
121
Issue:
14
Pages:
2753-2761
Publication date:
2013-01-01
Acceptance date:
2013-01-23
DOI:
EISSN:
1528-0020
ISSN:
0006-4971
Source identifiers:
508069

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