Letter to the EditorCysteine and hydrophobic residues in CDR3 serve as distinct T-cell self-reactivity indices
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Cited by (27)
HyperIgE in hypomorphic recombination-activating gene defects
2023, Current Opinion in ImmunologyCitation Excerpt :Low levels of recombination activity lead to the generation of a more restricted T- and B-cell repertoire with severe and early-onset clinical manifestations, typically observed in OS patients. Defective negative selection process in OS thymus leads to the egression of very few autoreactive thymocytes containing central cysteine and increased frequency of hydrophobic aminoacids at positions 6–7 of the CDR3 [31,32]. Dramatic reduction of T cells expressing the products of distal TCR alpha variable (TRAV) genes, including TRAV1-2, which are part of the semi-invariant TCR expressed by human mucosa-associated invariant T cells, was observed [33].
Poor T-cell receptor β repertoire diversity early posttransplant for severe combined immunodeficiency predicts failure of immune reconstitution
2022, Journal of Allergy and Clinical ImmunologyCitation Excerpt :Correlation was measured by Spearman rank correlation coefficient (Rs). Statistical analysis was performed using Prism Software or custom script (R environment, version 3.3.2) to calculate the Cysteine index.13 Severe restriction of repertoire diversity as measured by the Shannon [H] and the Simpson [1-D] indices and clonotypic expansions were observed 3 months after cellular therapy, followed by significant improvement by 6 months (P < .01) (Fig 1, A-C).
SASH3 variants cause a novel form of X-linked combined immunodeficiency with immune dysregulation
2021, BloodCitation Excerpt :Moreover, decreased diversity of the T-cell repertoire (Figure 5B) and prominent clonotypic expansions (Figure 5C) were observed in both patients compared with age-matched controls. Because autoimmune cytopenia was a prominent clinical manifestation in our cohort, we analyzed the frequency of TRA clonotypes containing hydrophobic amino acids at positions 6 and 7 of the CDR3, a molecular biomarker of self-reactivity.13 A higher proportion of such clonotypes was detected in both patients as compared with controls (Figure 5D).
Efficacy and safety of anti-CD45–saporin as conditioning agent for RAG deficiency
2021, Journal of Allergy and Clinical ImmunologySevere combined immune deficiency
2020, Stiehm's Immune Deficiencies: Inborn Errors of Immunity
Supported by the Monash Biomedicine Discovery Institute, the National Health and Medical Research Council (grant 1107464), and the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland.
Disclosure of potential conflict of interest: L. D. Notarangelo is supported by the National Institute of Allergy and Infectious Diseases; has board memberships with the Journal of Clinical Immunology, Clinical Immunology, and Frontiers in Immunology; and has received royalties from UpToDate. The rest of the authors declare that they have no relevant conflicts of interest.