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

Journal article

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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Authors: Gargiulo, L; Papaioannou, M; Sica, M; Talini, G; Chaidos, A

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: American Society of Hematology
• Journal: Blood
• Volume: 121
• Issue: 14
• Pages: 2753-2761
• Publication date: 2013-01-01
• Acceptance date: 2013-01-23
• DOI: 10.1182/blood-2012-11-469353
• EISSN: 1528-0020
• ISSN: 0006-4971
• Language: English
• Keywords: Middle Aged; Flow Cytometry; Adult; CD8-Positive T-Lymphocytes; Receptors, Antigen, T-Cell, alpha-beta; Humans; Dimerization; K562 Cells; Aged; Glycosylphosphatidylinositols; Gene Library; Young Adult; Antigen-Presenting Cells; Female; Male; Hemoglobinuria, Paroxysmal; Coculture Techniques; Antigens, CD1d; RNA, Messenger