Results 11 to 20 of about 1,349 (113)

Expression of Bruton's tyrosine kinase in B lymphoblastoid cell lines from X-linked agammaglobulinaemia patients

Clinical and Experimental Immunology, 1997
SUMMARY X-linked agammaglobulinaemia (XLA) is an immunodeficiency caused by mutations in Bruton's tyrosine kinase (Btk) and is characterized by an almost complete arrest of B cell development. We analysed expression of Btk in B lymphoblastoid cell lines (BLCL) derived from four unrelated XLA patients.
Weers, M (Michel), Dingjan, Gemma, Brouns, GS, Kraakman, MEM, Mensink, RGJ, Lovering, RC, Schuurman, RKB (Ruud), Borst, J, Hendriks, Rudi   +8 more
core   +6 more sources

Mutación de novo en el gen BTK en agammaglobulinemia ligada a X. Reporte de un caso del estado Mérida, Venezuela

Avances en Biomedicina, 2013
Las inmunodeficiencias primarias (IDPs) son un conjunto de enfermedades caracterizadas por defectos en el desarrollo y/o función del sistema inmune debido a anomalías genéticas en cualquiera de sus componentes.
Liliana Aboultaif Aboultaif, Lubna Nasr El Nimer, Maritza Navas, Lérida Margarita Borges Correa, José Angel Cova, Silvia Danelian, Morella Bouchard Pereira   +6 more
doaj   +3 more sources

B cell signalling pathways—New targets for precision medicine in chronic lymphocytic leukaemia

Scandinavian Journal of Immunology, Volume 92, Issue 5, November 2020., 2020
Abstract The B cell receptor (BCR) is a master regulator of B cells, controlling cellular processes such as proliferation, migration and survival. Cell signalling downstream of the BCR is aberrantly activated in the B cell malignancy chronic lymphocytic leukaemia (CLL), supporting the pathophysiology of the disease.
Sigrid S. Skånland, Linda Karlsen, Kjetil Taskén   +2 more
wiley   +1 more source

Inhibition of Bruton's TK regulates macrophage NF‐κB and NLRP3 inflammasome activation in metabolic inflammation

British Journal of Pharmacology, Volume 177, Issue 19, Page 4416-4432, October 2020., 2020
Background and Purpose There are no medications currently available to treat metabolic inflammation. Bruton's tyrosine kinase (BTK) is highly expressed in monocytes and macrophages and regulates NF‐κB and NLRP3 inflammasome activity; both propagate metabolic inflammation in diet‐induced obesity.
Gareth S.D. Purvis, Massimo Collino, Haidee Aranda‐Tavio, Fausto Chiazza, Caroline E. O'Riordan, Lynda Zeboudj, Shireen Mohammad, Debora Collotta, Roberta Verta, Nicolas E.S. Guisot, Peter Bunyard, Magdi M. Yaqoob, David R. Greaves, Christoph Thiemermann   +13 more
wiley   +1 more source

Persisting SARS‐CoV‐2 viraemia after rituximab therapy: two cases with fatal outcome and a review of the literature

British Journal of Haematology, Volume 190, Issue 2, Page 185-188, July 2020., 2020
Summary SARS‐CoV‐2 infection can cause severe pneumonia (COVID‐19). There is evidence that patients with comorbidities are at higher risk of a severe disease course. The role of immunosuppression in the disease course is not clear. In the present report, we first describe two cases of persisting SARS‐CoV‐2 viraemia with fatal outcome in patients after ...
Phil‐Robin Tepasse, Wali Hafezi, Mathias Lutz, Joachim Kühn, Christian Wilms, Rainer Wiewrodt, Jan Sackarnd, Martin Keller, Hartmut H. Schmidt, Richard Vollenberg   +9 more
wiley   +1 more source

Ecthyma gangrenosum as the presenting clinical feature of X-linked agammaglobulinaemia: report of three cases and a review of literature [PDF]

, 2021
Background: Children with X-linked agammaglobulinaemia (XLA) usually present with pneumonia and otitis media caused by pyogenic bacteria. Rarely, ecthyma gangrenosum (EG), a known cutaneous manifestation of Pseudomonas septicaemia present in XLA as the ...
Adam, Julia, Ramlan, Saidatul Saadah, Mat Ripen, Adiratna, Ismail, Intan Hakimah, Mohd Radzi, Farah Syuhada, Zainal Abidin, Mohd Azri   +5 more
core   +1 more source

Bruton's tyrosine kinase expression and activity in X-linked agammaglobulinaemia (XLA): the use of protein analysis as a diagnostic indicator of XLA [PDF]

Clinical and Experimental Immunology, 1998
SUMMARY Mutations in the Bruton's tyrosine kinase (BTK) gene result in XLA. Despite the large numbers of BTK mutations reported, no correlation can be made between the clinical phenotype and the gene defects. Analysis of Btk protein expression and activity in individuals with XLA was performed to characterize the relationship between a ...
H B, Gaspar, T, Lester, R J, Levinsky, C, Kinnon   +3 more
openaire   +2 more sources

Autologous non‐human primate model for safety assessment of piggyBac transposon‐mediated chimeric antigen receptor T cells on granulocyte–macrophage colony‐stimulating factor receptor

Clinical &Translational Immunology, Volume 9, Issue 11, 2020., 2020
We pre‐clinically evaluated the safety of granulocyte–macrophage colony‐stimulating factor (GMCSF) receptor (GMR)–chimeric antigen receptor (CAR) T cells. For this purpose, we used a novel immunocompetent non‐human primate (NHP) model, established using bioengineered cynomolgus T cells expressing CAR‐T molecules redirected against the GMR by piggyBac ...
Hirokazu Morokawa, Shigeki Yagyu, Aiko Hasegawa, Miyuki Tanaka, Shoji Saito, Hidemi Mochizuki, Kengo Sakamoto, Akihito Shimoi, Yozo Nakazawa   +8 more
wiley   +1 more source

Phylogeny of Tec Family Kinases: Identification of a Pre-Metazoan Origin of Btk, Bmx, Itk, Tec, Txk and the Btk Regulator SH3BP5 [PDF]

, 2008
It is generally considered mammals and birds have five Tec family kinases (TFKs): Btk, Bmx (also known as Etk), Itk, Tec, and Txk (also known as Rlk). Here, we discuss the domains and their functions and regulation in TFKs.
Ortutay, Csaba, Beston F. Nore, Nore, Beston F., Vihinen, Mauno, C.I. Edvard Smith, Smith, C. I. Edvard, Csaba Ortutay, Mauno Vihinen   +7 more
core   +1 more source

Impaired polysaccharide responsiveness without agammaglobulinaemia in three patients with hypomorphic mutations in Bruton Tyrosine Kinase—No detection by newborn screening for primary immunodeficiencies [PDF]

Scandinavian Journal of Immunology, 2019
AbstractHypomorphic mutations in the gene encoding Bruton tyrosine kinase (BTK) may result in milder phenotypes and delayed diagnosis of B‐cell related immunodeficiencies due to residual BTK function. Newborn screening for kappa‐deleting‐recombination‐excision circles (KRECs) reliably identifies classical X‐linked agammaglobulinaemia (XLA) patients ...
Renate Krüger, Ulrich Baumann, Stephan Borte, Uwe Kölsch, Myriam Ricarda Lorenz, Baerbel Keller, Ina Harder, Klaus Warnatz, Stephan Ehl, Klaus Schwarz, Volker Wahn, Horst von Bernuth   +11 more
openaire   +3 more sources