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Impaired B-cell function in deficiency.
Front Immunol
July 2024
Immunology Outpatient Clinic, Vienna, Austria.
Article Synopsis
- Trichothiodystrophy-1 (TTD1) is an autosomal-recessive disease linked to mutations in a gene important for DNA repair and transcription, leading to increased susceptibility to infections in affected patients.
- The study examined the immune system and DNA repair capabilities of three TTD1 patients, revealing issues such as low antibody responses, problems with B-cell proliferation, and accumulation of DNA damage markers.
- Findings indicated that TTD1 is associated with antibody deficiencies due to impaired B-cell development and function, confirming the impact of new mutations on the immune system.
ETS1 phosphorylation at threonine 38 is associated with the cell of origin of diffuse large B cell lymphoma and sustains the growth of tumour cells.
Br J Haematol
October 2023
Institute of Oncology Research, Faculty of Biomedical Sciences, USI, Bellinzona, Switzerland.
The transcriptional factor ETS1 is upregulated in 25% of diffuse large B cell lymphoma (DLBCL). Here, we studied the role of ETS1 phosphorylation at threonine 38, a marker for ETS1 activation, in DLBCL cellular models and clinical specimens. p-ETS1 was detected in activated B cell-like DLBCL (ABC), not in germinal centre B-cell-like DLBCL (GCB) cell lines and, accordingly, it was more common in ABC than GCB DLBCL diagnostic biopsies.
View Article and Find Full Text PDFDevelopment and characterization of a CRISPR/Cas9-mediated knockout chicken model lacking mature B and T cells.
Front Immunol
August 2022
Department of Agricultural Biotechnology and Research Institute of Agriculture and Life Sciences, College of Agriculture and Life Sciences, Seoul National University, Seoul, South Korea.
Although birds have been used historically as a model animal for immunological research, resulting in remarkable achievements, immune cell development in birds themselves has yet to be fully elucidated. In this study, we firstly generated an immunodeficient chicken model using a CRISPR/Cas9-mediated recombination activating gene 1 () knockout, to investigate avian-specific immune cell development. Unlike previously reported immunoglobulin (Ig) heavy chain knockout chickens, the proportion and development of B cells in both and embryos were significantly impaired during B cell proliferation (embryonic day 16 to 18).
View Article and Find Full Text PDFCEACAM1 specifically suppresses B cell receptor signaling-mediated activation.
Biochem Biophys Res Commun
January 2021
Department of Advanced Therapeutics for GI Diseases, Graduate School of Medical Science, TMDU, Tokyo, Japan. Electronic address:
Carcinoembryonic antigen-related cell adhesion molecule 1 (CEACAM1) expressed in T cells may regulate immune responses in the gut. In addition to T cells, B cells are also an important population in the gut-associated lymphoid tissues that orchestrate mucosal homeostasis. However, the role of CEACAM1 in B cells has not been elucidated.
View Article and Find Full Text PDFBCALM (AC099524.1) Is a Human B Lymphocyte-Specific Long Noncoding RNA That Modulates B Cell Receptor-Mediated Calcium Signaling.
J Immunol
August 2020
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110
Of the thousands of long noncoding RNAs (lncRNA) identified in lymphocytes, very few have defined functions. In this study, we report the discovery and functional elucidation of a human B cell-specific lncRNA with high levels of expression in three types of B cell cancer and normal B cells. The gene is upstream of the gene encoding the B cell-specific phospholipase C γ 2 (), a B cell-specific enzyme that stimulates intracellular Ca signaling in response to BCR activation.
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