ArticleJournal of inherited metabolic disease2012
Aberrant expression of costimulatory molecules in splenocytes of the mevalonate kinase-deficient mouse model of human hyper-IgD syndrome (HIDS).
Article in Journal of inherited metabolic disease, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
What it found
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Who cites it
4 citing papers in PubMed, 9 citations in OpenAlex.
- Mevalonate kinase deficiency: current perspectives.The application of clinical genetics · 2016Review
- Hyper-IgD syndrome/mevalonate kinase deficiency: what is new?Seminars in immunopathology · 2015Review
- Long chain fatty acid (Lcfa) abnormalities in hyper Igd syndrome (Hids) and Familial Mediterranean Fever (Fmf): new insight into heritable periodic fevers.Molecular genetics and metabolism · 2013Article
- 30 years hids-Travels between bedside and bench.Temperature (Austin, Tex.)Article
Corrections and comments
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Authors and funding
4 authors at 4 institutions in 1 country.
Funding
Abstract
objectiveWe sought to determine the activation status and proliferative capacities of splenic lymphocyte populations from a mevalonate kinase-deficient mouse model of hyper-IgD syndrome (HIDS). We previously reported that murine mevalonate kinase gene ablation was embryonic lethal for homozygous mutants while heterozygotes (Mvk (+/-)) demonstrated several phenotypic features of human HIDS including increased serum levels of IgD, IgA, and TNFα, temperature dysregulation, hematological abnormalities, and splenomegaly. METHODS AND
resultsFlow cytometric analysis of cell surface activation markers on T and B lymphocytes, and macrophage populations, demonstrated aberrant expression of B7 glycoproteins in all splenic cell types studied. Differences in expression levels between Mvk (+/-) and Mvk (+/+) littermate controls were observed in both the basal state (unstimulated) and after Concanavalin A (Con-A) stimulation in vitro of whole splenocyte cultures. In Mvk (+/-) CD4 and CD8 T cells, alterations in expression of CD25, CD80, CD152, and CD28 were observed. Mvk (+/-) splenic macrophages expressed altered levels of CD80, CD86, CD40, and CD11c while Mvk (+/-) B lymphocytes had differential expression of CD40, CD80, and CD86. Mvk (+/-) splenocyte subpopulations also exhibited altered proliferative capacities in response to in vitro stimulation.
conclusionWe postulate that imbalances in the expression of cell surface proteins necessary for activation, proliferation, and regulation of the intensity and duration of an immune response may result in defective T cell activation, proliferation, and effector functions in our model and potentially in human HIDS.
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Registered trials
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