Phylogeny of Tec Family Kinases: Identification of a Pre-Metazoan Origin of Btk, Bmx, Itk, Tec, Txk and the Btk Regulator SH3BP5 [PDF]
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.
Nore, Beston F.+3 more
core +1 more source
Targeting Brutons Tyrosine Kinase in Chronic Lymphocytic Leukemia at the Crossroad between Intrinsic and Extrinsic Pro-survival Signals [PDF]
Chemo immunotherapies for chronic lymphocytic leukemia (CLL) showed a positive impact on clinical outcome, but many patients relapsed or become refractory to the available treatments.
Facco, Monica+7 more
core +1 more source
We describe the first case of infection with Helicobacter trogontum in a patient with X-linked agammaglobulinemia. A 22-year-old male with X-linked agammaglobulinemia presented with fever, malaise and a painful skin lesion on the lower left extremity ...
Lasse Fjordside+4 more
doaj +1 more source
Subcutaneous Immunoglobulin Replacement Therapy with Hizentra® is Safe and Effective in Children Less Than 5 Years of Age. [PDF]
BACKGROUND:Hizentra® (IGSC 20%) is a 20% liquid IgG product approved for subcutaneous administration in adults and children 2 years of age and older who have primary immunodeficiency disease (PIDD). There is limited information about the use of IGSC 20 %
Atkinson, Thomas Prescott+10 more
core +2 more sources
Mechanism-based strategies for the management of autoimmunity and immune dysregulation in primary immunodeficiencies [PDF]
A broad spectrum of autoimmunity is now well described in patients with primary immunodeficiencies (PIDs). Management of autoimmune disease in the background of PID is particularly challenging given the seemingly discordant goals of immune support and ...
Cooper, Megan A+4 more
core +2 more sources
Late diagnosis of agammaglobulinemia in an 8-year-old boy
Chromosome X-linked Bruton agammaglobulinemia is classified as a primary immunodeficiency disorder. It is a genetic condition associated with a mutation in the BTK gene encoding tyrosine kinase.
Małgorzata Sopińska+4 more
doaj +1 more source
Subcutaneous IgG Replacement Therapy by Push in 32 Patients with Primary Immunodeficiency Diseases in Argentine [PDF]
Introduction: Regular replacement with immunoglobulin infusions is the mainstay of treatment in the majority of primary immunodeficiencies. Several studies showed that Subcutaneous Immunoglobulin (SCIG) has similar efficacy to Intravenous Immunoglobulin (
Bezrodnik, Liliana+6 more
core +1 more source
Fatal SARS in X-Linked Lymphoproliferative Disease Type 1: A Case Report
X-linked lymphoproliferative disease (XLP1) is an inborn error of immunity (IEI) with severe immune dysregulation caused by a mutation in the SH2D1A gene resulting in the absence or dysfunction of signaling lymphocytic activation molecule (SLAM ...
Ming Hin Chung+5 more
doaj +1 more source
Severe B Cell Deficiency in Mice Lacking the Tec Kinase Family Members Tec and Btk [PDF]
The cytoplasmic protein tyrosine kinase Tec has been proposed to have important functions in hematopoiesis and lymphocyte signal transduction. Here we show that Tec-deficient mice developed normally and had no major phenotypic alterations of the immune ...
Baltimore, David+7 more
core +3 more sources
Presentation of a case of Bruton type primary agammaglobulinemia in Guinea
X-linked agammaglobulinemia (XLA) is a rare genetic disease caused by a mutation in the Bruton tyrosine kinase (BTK) gene. It is characterized by a profound deficiency of B cells and a decrease in all classes of immunoglobulins (Ig).
Kaba Condé+4 more
doaj +1 more source