Objective. To elucidate the role played by HLA-B51 in the neutrophil hyperfunction of Behqet's disease, we determined the superoxide production by purified peripheral blood neutrophils from Behqet's disease patients, from HLA-B51 positive healthy individuals, and from HLA-B51 transgenic mice, Methods. Neutrophil function was evaluated by flow cytometric analysis, detecting the conversion of 2',7'-dichlorofluorescin diacetate into dichlorofluorescein, induced by superoxide in the neutrophils.Results. A significant correlation between the neutrophil hyperfunction and the possession of HLA-B51 phenotype, regardless of the presence of the disease, was observed in humans. FMLP-stimulated neutrophils (without in vitro priming) from HLA-BS1 transgenic mice, but not those from HLA-B35 transgenic mice or from nontransgenic mice, produced substantial amounts of superoxide.Conclusion. The HLA-BS1 molecule itself may be responsible, at least in part, for neutrophil hyperfunction in Behqet's disease.
Phenotypic and functional properties of γδ T cells, which play an important role in mucocutaneous immunity, were examined to elucidate whether immunological abnormality in Behc¸et's disease may be related to a specific T cell population. We found that CD45RA+Vγ9+Vδ2+γδ T cells, which constitute a minor population of γδ T cells in healthy individuals, were increased in number in Behc¸et's disease irrespective of disease activity. This CD45RA+ subset of γδ T cells in the active, but not inactive, phase of this disease expressed IL‐2Rβ and HLA‐DR, suggesting that they are activated in vivo in active Behc¸et's disease. In addition, the CD45RA+γδ T cells produced extreme amounts of tumour necrosis factor and contained perforin granules. These data indicate that a phenotypically distinct subset of γδ T cells, CD45RA+CD45RO−Vγ9+Vδ2+, may contribute to immunological abnormalities which may lead to complexity of pathophysiology in Behc¸et's disease.
Objective. We have recently found that the presence of autoantibodies against the 70-kd polypeptide of U1 RNP (Ul-70-kd) is associated with HLA-DR4 and DR2. To further characterize this association, we performed a molecular genetic analysis of HLA-DR and DQ genes among patients with autoantibodies against Methods. The polymerase chain reaction (PCR), sequence-specific oligonucleotide hybridization, and U1-70-kd.
In this study, we induced differentiation of CPT II-deficient hiPSCs into mature myocytes in a highly efficient and reproducible manner and recapitulated some aspects of the disease phenotypes of CPT II deficiency in the myocyte disease models. This approach addresses the challenges of modeling the abnormality of FAO in CPT II deficiency using iPSC technology and has the potential to revolutionize translational research in this field.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.