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Publication Detail
CD19+CD24hiCD38hi B Cells Are Expanded in Juvenile Dermatomyositis and Exhibit a Pro-Inflammatory Phenotype After Activation Through Toll-Like Receptor 7 and Interferon-α
  • Publication Type:
    Journal article
  • Publication Sub Type:
  • Authors:
    Piper CJM, Wilkinson MGL, Deakin CT, Otto GW, Dowle S, Duurland CL, Adams S, Marasco E, Rosser EC, Radziszewska A, Carsetti R, Ioannou Y, Beales PL, Kelberman D, Isenberg DA, Mauri C, Nistala K, Wedderburn LR, Armon K, Ellis-Gage J, Roper H, Briggs V, Watts J, McCann L, Roberts I, Baildam E, Hanna L, Lloyd O, Wadeson S, Riley P, McGovern A, Ryder C, Scott J, Thomas B, Southwood T, Al-Abadi E, Wyatt S, Jackson G, Amin T, Wood M, VanRooyen V, Burton D, Davidson J, Gardner-Medwin J, Martin N, Ferguson S, Waxman L, Browne M, Friswell M, Foster H, Swift A, Jandial S, Stevenson V, Wade D, Sen E, Smith E, Qiao L, Watson S, Duong C, Venning H, Satyapal R, Stretton E, Jordan M, Mosley E, Frost A, Crate L, Warrier K, Stafford S, Wedderburn L, Pilkington C, Hasson N, Maillard S, Halkon E, Brown V, Juggins A, Smith S, Lunt S, Enayat E, Varsani H, Kassoumeri L, Beard L, Arnold K, Glackin Y, Simou S, Almeida B, Marques R
  • Publication date:
  • Journal:
    Frontiers in Immunology
  • Volume:
  • Issue:
  • Status:
© 2018 Piper, Wilkinson, Deakin, Otto, Dowle, Duurland, Adams, Marasco, Rosser, Radziszewska, Carsetti, Ioannou, Beales, Kelberman, Isenberg, Mauri, Nistala and Wedderburn. Juvenile dermatomyositis (JDM) is a rare form of childhood autoimmune myositis that presents with proximal muscle weakness and skin rash. B cells are strongly implicated in the pathogenesis of the disease, but the underlying mechanisms are unknown. Therefore, the main objective of our study was to investigate mechanisms driving B cell lymphocytosis and define pathological features of B cells in JDM patients. Patients were recruited through the UK JDM Cohort and Biomarker study. Peripheral blood B cell subpopulations were immunophenotyped by flow cytometry. The results identified that immature transitional B cells were significantly expanded in active JDM, actively dividing, and correlated positively with disease activity. Protein and RNAseq analysis revealed high interferon alpha (IFNa) and TLR7-pathway signatures pre-treatment. Stimulation of B cells through TLR7/8 promoted both IL-10 and IL-6 production in controls but failed to induce IL-10 in JDM patient cells. Interrogation of the CD40-CD40L pathway (known to induce B cell IL-10 and IL-6) revealed similar expression of IL-10 and IL-6 in B cells cultured with CD40L from both JDM patients and controls. In conclusion, JDM patients with active disease have a significantly expanded immature transitional B cell population which correlated with the type I IFN signature. Activation through TLR7 and IFNa may drive the expansion of immature transitional B cells in JDM and skew the cells toward a pro-inflammatory phenotype.
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Genetics & Genomic Medicine Dept
Div of Medicine
Div of Infection & Immunity
Div of Infection & Immunity
Infection, Immunity & Inflammation Dept
Infection, Immunity & Inflammation Dept
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