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Cited by 89T-cell and B-cell ImmunologyBlood groups and transfusionComplement system in diseases

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

Gargiulo, Lucia, Papaioannou, Maria, Sica, Michela, Talini, Giulia, Chaidos, Aristeidis, Richichi, Barbara, Nikolaev, Andrei V., Nativi, Cristina +6 more·2013·Blood
DOI 10.1182/blood-2012-11-469353
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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 demonstrated in PNH patients the presence of CD8(+) T cells reactive against antigen-presenting cells (APCs) loaded with GPI. These T cells were significantly more abundant in PNH patients than in healthy controls; their reactivity depended on CD1d expression and they increased upon coculture with CD1d-expressing, GPI-positive APCs. In GPI-specific T cells captured by CD1d dimer technology, we identified, through global T-cell receptor α (TCRα) analysis, an invariant TCRVα21 sequence, which was then found at frequencies higher than background in the TCR repertoire of 6 of 11 PNH patients. Thus, a novel, autoreactive, CD1d-restricted, GPI-specific T-cell population, enriched in an invariant TCRα chain, is expanded in PNH patients and may be responsible for BMF in PNH.

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Crossref · 10.1182/blood-2012-11-469353

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  • A CD1-restricted T-cell response can lead to elimination of hematopoietic cells while creating a selective advantage for clones with deficiency for phosphatidylinositol glycan biosynthesis class A protein, reported in patients with paroxysmal nocturnal hemoglobinuria (PNH).

    “A CD1-restricted T-cell response can lead to an elimination of hematopoietic cells, while at the same time creating a selective advantage for clones with deficiency for phosphatidylinositol glycan biosynthesis class A protein, reported in patients with paroxysmal nocturnal hemoglobinuria (PNH).63”

    From Inflammatory bone marrow microenvironment (2019) · cites this paper as [63]

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Authors
Gargiulo, L., Papaioannou, M., Sica, M., Talini, G., Chaidos, A., Richichi, B., Nikolaev, A. V., Nativi, C., Layton, M., de la Fuente, J., Roberts, I., Luzzatto, L., Notaro, R., & Karadimitris, A.
Affiliations
  • Hammersmith Hospital (GB)
  • Cancer Genetics and Gene Transfer-Core Research Laboratory, Istituto Toscano Tumori, Firenze, Italy;
  • University of Florence (IT)
  • University of Dundee (GB)
  • Paediatric Haematology, Blood and Marrow Transplant Programme, St. Mary’s Hospital, Imperial College Healthcare National Health Service (NHS) Trust, London, United Kingdom; and
  • Tumour Institute of Tuscany (IT)
ORCID
Venue
Blood
Publisher
American Society of Hematology
Published
2013-04-04
Volume
121
Issue
14
Pages
2753-2761
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
DOI
10.1182/blood-2012-11-469353
OpenAlex
W1974953236
Cited by
89
References
41
Citations by year
2026: 4 · 2025: 1 · 2024: 3 · 2023: 7 · 2022: 6 · 2021: 7 · 2020: 9 · 2019: 9 · 2018: 10 · 2017: 13 · 2016: 7 · 2015: 6 · 2014: 5 · 2013: 2
FWCI
3.68
Type
journal article
Language
en
Open access
closed
Locations
  • Blood (publishedVersion)
  • PubMed (publishedVersion)
  • Discovery Research Portal (University of Dundee) (submittedVersion)
  • Florence Research (University of Florence) (submittedVersion)
Topics
  • Life Sciences › Immunology and Microbiology › Immunology › T-cell and B-cell Immunology
  • Health Sciences › Medicine › Hematology › Blood groups and transfusion
  • Life Sciences › Immunology and Microbiology › Immunology › Complement system in diseases
Keywords
paroxysmal nocturnal hemoglobinuria, glycosylphosphatidylinositol, CD1d-restricted T cells, CD8+ T cells, bone marrow failure, PIG-A mutation, autoreactive T cells, T cell receptor alpha chain, antigen-presenting cells, autoimmunity
MeSH terms
Bone Marrow Failure Disorders, Adult, Aged, Anemia, Aplastic, Antigen-Presenting Cells, Bone Marrow Diseases, Female, Flow Cytometry, Hemoglobinuria, Paroxysmal, Humans, Male, Middle Aged, RNA, Messenger, Gene Library, Receptors, Antigen, T-Cell, alpha-beta, Glycosylphosphatidylinositols, CD8-Positive T-Lymphocytes, Coculture Techniques, Dimerization, K562 Cells, Antigens, CD1d, Young Adult

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