Adamska, Julia Z Zia, Amin Bloom, Michelle S Crofford, Leslie J Furst, Daniel E Goldmuntz, Ellen Keyes-Elstein, Lynette Mayes, Maureen D McSweeney, Peter Nash, Richard A
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Published in
Annals of the rheumatic diseases
Myeloablative autologous haematopoietic stem cell transplant (HSCT) was recently demonstrated to provide significant benefit over cyclophosphamide (CYC) in the treatment of diffuse cutaneous systemic sclerosis (dcSSc) in the Scleroderma: Cyclophosphamide or Transplantation (SCOT) trial. As dysregulation of the B cell compartment has previously been...
Rivière, Elodie Mariette, Xavier Nocturne, Gaetane
Published in
Annals of the rheumatic diseases
Wray, Jason P Deltcheva, Elitza M Boiers, Charlotta Richardson, Simon Е Chhetri, Jyoti Bikram Brown, John Gagrica, Sladjana Guo, Yanping Illendula, Anuradha Martens, Joost HA
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Funder: Blood Cancer UK/Grant Number 16001 / The ETV6-RUNX1 onco-fusion arises in utero, initiating a clinically silent pre-leukemic state associated with the development of pediatric B-acute lymphoblastic leukemia (B-ALL). We characterize the ETV6-RUNX1 regulome by integrating chromatin immunoprecipitation- and RNA-sequencing and show that ETV6-RU...
Qin, Yi Cai, Ming-Long Jin, Hui-Zhi Huang, Wei Zhu, Chen Bozec, Aline Huang, Jingang Chen, Zhu
Published in
Annals of the rheumatic diseases
Age-associated B cells (ABCs) are a recently identified B cell subset, whose expansion has been increasingly linked to the pathogenesis of autoimmune disorders. This study aimed to investigate whether ABCs are involved in the pathogenesis and underlying mechanisms of rheumatoid arthritis (RA). ABCs were assessed in collagen-induced arthritis (CIA) ...
Bos, Amélie V van Gool, Melissa MJ Breedveld, Annelot C van der Mast, Richard Marsman, Casper Bouma, Gerd van de Wiel, Mark A van Ham, S Marieke Mebius, Reina E van Egmond, Marjolein
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Patients with inflammatory bowel disease (IBD) produce enhanced immunoglobulin A (IgA) against the microbiota compared to healthy individuals, which has been correlated with disease severity. Since IgA complexes can potently activate myeloid cells via the IgA receptor FcαRI (CD89), excessive IgA production may contribute to IBD pathology. However, ...
Li, Qi Yu, Jieyu Zhang, Hao Meng, Yinghao Liu, Yan Fang Jiang, Hui Zhu, Mengmeng Li, Na Zhou, Jian Liu, Fang
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Published in
Academic radiology
Conventional chemotherapy has limited benefit in pancreatic ductal adenocarcinoma (PDAC), necessitating identification of novel therapeutic targets. Radiomics may enable non-invasive prediction of CD20 expression, a hypothesized therapeutic target in PDAC. To develop a machine learning classifier based on noncontrast magnetic resonance imaging for ...
Rae, William Sowerby, John M Verhoeven, Dorit Youssef, Mariam Kotagiri, Prasanti Savinykh, Natalia Coomber, Eve L Boneparth, Alexis Chan, Angela Gong, Chun
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Tumor necrosis factor receptor-associated factor 3 (TRAF3) is a central regulator of immunity. TRAF3 is often somatically mutated in B cell malignancies, but its role in human immunity is not defined. Here, in five unrelated families, we describe an immune dysregulation syndrome of recurrent bacterial infections, autoimmunity, systemic inflammation...
Machado, Heather E Mitchell, Emily Øbro, Nina F Kübler, Kirsten Davies, Megan Leongamornlert, Daniel Cull, Alyssa Maura, Francesco Sanders, Mathijs A Cagan, Alex TJ
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Funder: Wellcome Trust / The lymphocyte genome is prone to many threats, including programmed mutation during differentiation1, antigen-driven proliferation and residency in diverse microenvironments. Here, after developing protocols for expansion of single-cell lymphocyte cultures, we sequenced whole genomes from 717 normal naive and memory B and ...
Csomos, Krisztian Ujhazi, Boglarka Blazso, Peter Herrera, Jose L Tipton, Christopher M Kawai, Tomoki Gordon, Sumai Ellison, Maryssa Wu, Kevin Stowell, Matthew
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The recombination-activating genes (RAG) 1 and 2 are indispensable for diversifying the primary B cell receptor repertoire and pruning self-reactive clones via receptor editing in the bone marrow; however, the impact of RAG1/RAG2 on peripheral tolerance is unknown. Partial RAG deficiency (pRD) manifesting with late-onset immune dysregulation repres...
Madissoon, Elo Oliver, Amanda J Kleshchevnikov, Vitalii Wilbrey-Clark, Anna Polanski, Krzysztof Richoz, Nathan Ribeiro Orsi, Ana Mamanova, Lira Bolt, Liam Elmentaite, Rasa
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Single-cell transcriptomics has allowed unprecedented resolution of cell types/states in the human lung, but their spatial context is less well defined. To (re)define tissue architecture of lung and airways, we profiled five proximal-to-distal locations of healthy human lungs in depth using multi-omic single cell/nuclei and spatial transcriptomics ...
