Dendritic and T Cell Response to Influenza is Normal in the Patients with X-Linked Agammaglobulinemia [PDF]
Introduction Influenza virus is a potential cause of severe disease in the immunocompromised. X-linked agammaglobu-linemia (XLA) is a primary immunodeficiency characterized by the lack of immunoglobulin, B cells, and plasma cells, secondary to mutation ...
A Amedei +41 more
core +3 more sources
Accelerated Immunodeficiency-associated Vaccine-derived Poliovirus Serotype 3 Sequence Evolution Rate in an 11-week-old Boy With X-linked Agammaglobulinemia and Perinatal Human Immunodeficiency Virus Exposure [PDF]
Primary B-cell immunodeficiencies are risk factors for the generation of vaccine-derived polioviruses. We report immunodeficiency-associated vaccine-derived poliovirus serotype 3 in an 11-week-old boy with X-linked agammaglobulinemia.
Berkowitz, N +21 more
core +1 more source
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 +6 more
doaj
Expression and regulation of antimicrobial peptides in mucosal immunity [PDF]
Antimicrobial polypeptides (AMPs) are effector molecules of the innate immune defense. AMPs are mainly expressed in epithelial cells and immune cells, providing the first line of defense to infection as direct antimicrobials.
Cederlund, Andreas
core +1 more source
Profound reduction of mature B cell numbers, reactivities and serum lg levels in mice which simultaneously carry the XID and CD40 deficiency gense [PDF]
It has been known for some time that single mutant nude or CD40T mice have apparently normal numbers of cells in the precursor compartments of bone marrow and the mature B cell compartments of the periphery.
Andersson, Jan +8 more
core
B lymphocytes as effector cells in the immunotherapy of cancer [PDF]
Over the years, the role of B cells in the host immune response to malignancy has been overshadowed by our focus on T cells. Nevertheless, B cells play important roles as antigen‐presenting cells and in the production of antibodies.
Chang, Alfred E. +8 more
core +1 more source
Low dose Btk inhibitors selectively block platelet activation by CLEC-2 [PDF]
Inhibitors of the tyrosine kinase Btk have been proposed as novel antiplatelet agents. In this study we show that low concentrations of the Btk inhibitor ibrutinib block CLEC-2-mediated activation and tyrosine phosphorylation including Syk and PLCγ2 in ...
Brill, Alexander +19 more
core +5 more sources
Btk Mutations Selectively Regulate Btk Expression And Upregulate Monocyte Xbp1 Mrna In Xla Patients. [PDF]
Mutations in the Bruton agammaglobulinemia tyrosine kinase (BTK) gene are responsible for X-linked agammaglobulinemia (XLA). Unfolded or misfolded proteins can trigger stress pathways in the endoplasmic reticulum (ER), known as unfolded protein response (
Abramczuk, Beatriz M +4 more
core +1 more source
Recurrent pneumonia with mild hypogammaglobulinemia diagnosed as X-linked agammaglobulinemia in adults [PDF]
Background X-linked agammaglobulinemia (XLA) is a humoral immunodeficiency caused by disruption of the Bruton's tyrosine kinase (BTK) gene. Typical XLA patients suffer recurrent and severe bacterial infections in childhood.
Hagiwara, Koichi +7 more
core +3 more sources
Molecular studies on Burton's tyrosine kinase [PDF]
X-Linked agammaglobulinaemia (XLA) is a humoral immunodeficiency resulting from a block in the B cell maturation pathway at the pre-B cell stage. Affected males have normal numbers of pre-B cells in the bone marrow but virtually no mature B cells in the ...
Gaspar, Hubert Baburaj
core

