Review
doi: 10.1016/j.coi.2008.02.001.
Epub 2008 Apr 9.
Genetically determined lymphopenia and autoimmune manifestations
Affiliations
- PMID: 18403192
- DOI: 10.1016/j.coi.2008.02.001
Item in Clipboard
Review
Genetically determined lymphopenia and autoimmune manifestations
Curr Opin Immunol.
2008 Jun.
Abstract
Hypomorphic defects of V(D)J recombination in humans lead to residual T cell development. In these lymphopenic conditions, homeostatic lymphocyte proliferation occurs, and key mechanisms that normally maintain host tolerance are altered, allowing peripheral expansion of oligoclonal and autoreactive T cells. Recently described murine models support this notion. This review describes human and murine situations, in which genetically determined T and B cell lymphopenia is associated with autoimmune manifestations.
Similar articles
-
Of Omenn and mice.Trends Immunol. 2008 Mar;29(3):133-40. doi: 10.1016/j.it.2007.12.001. Epub 2008 Feb 5. Trends Immunol. 2008. PMID: 18255337 Review.
-
Autoimmunity during lymphopenia: a two-hit model.Clin Immunol. 2006 Aug;120(2):121-8. doi: 10.1016/j.clim.2006.04.569. Clin Immunol. 2006. PMID: 16766227 Review.
-
PTPN22 Acts in a Cell Intrinsic Manner to Restrict the Proliferation and Differentiation of T Cells Following Antibody Lymphodepletion.Front Immunol. 2020 Jan 28;11:52. doi: 10.3389/fimmu.2020.00052. eCollection 2020. Front Immunol. 2020. PMID: 32047502 Free PMC article.
-
Highly variable clinical phenotypes of hypomorphic RAG1 mutations.Pediatrics. 2010 Nov;126(5):e1248-52. doi: 10.1542/peds.2009-3171. Epub 2010 Oct 18. Pediatrics. 2010. PMID: 20956421
-
Lymphopenia-induced proliferation is a potent activator for CD4+ T cell-mediated autoimmune disease in the retina.J Immunol. 2009 Jan 15;182(2):969-79. doi: 10.4049/jimmunol.182.2.969. J Immunol. 2009. PMID: 19124740 Free PMC article.
Cited by
-
Breakdown of T cell tolerance and autoimmunity in primary immunodeficiency--lessons learned from monogenic disorders in mice and men.Curr Opin Immunol. 2008 Dec;20(6):646-54. doi: 10.1016/j.coi.2008.10.004. Epub 2008 Nov 12. Curr Opin Immunol. 2008. PMID: 18955138 Free PMC article. Review.
-
Noncore RAG1 regions promote Vβ rearrangements and αβ T cell development by overcoming inherent inefficiency of Vβ recombination signal sequences.J Immunol. 2014 Feb 15;192(4):1609-19. doi: 10.4049/jimmunol.1301599. Epub 2014 Jan 10. J Immunol. 2014. PMID: 24415779 Free PMC article.
-
Mechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies.J Allergy Clin Immunol Pract. 2016 Nov-Dec;4(6):1089-1100. doi: 10.1016/j.jaip.2016.08.004. J Allergy Clin Immunol Pract. 2016. PMID: 27836058 Free PMC article. Review.
-
New insights into T cell biology and T cell-directed therapy for autoimmunity, inflammation, and immunosuppression.Ann N Y Acad Sci. 2010 Jan;1183:123-48. doi: 10.1111/j.1749-6632.2009.05124.x. Ann N Y Acad Sci. 2010. PMID: 20146712 Free PMC article. Review.
-
Potential therapeutic applications of antisense morpholino oligonucleotides in modulation of splicing in primary immunodeficiency diseases.J Immunol Methods. 2011 Feb 28;365(1-2):1-7. doi: 10.1016/j.jim.2010.12.001. Epub 2010 Dec 13. J Immunol Methods. 2011. PMID: 21147113 Free PMC article. Review.
Publication types
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
Medical
