"RIV/00843989:_____/12:00102611!RIV13-MZ0-00843989" . "Basofily a \u017E\u00EDrn\u00E9 bu\u0148ky p\u016Fsob\u00ED jako regula\u010Dn\u00ED a efektorov\u00E9 bu\u0148ky p\u0159irozen\u00FDch imunitn\u00EDch mechanism\u016F a v sou\u010Dasn\u00E9 dob\u011B jsou pova\u017Eov\u00E1ny za spojnici mezi vrozenou a adaptivn\u00ED imunitou. Spolu s \u017E\u00EDrn\u00FDmi bu\u0148kami se pod\u00EDlej\u00ED na vzniku alergick\u00E9 reakce okam\u017Eit\u00E9ho typu zprost\u0159edkovan\u00E9 IgE. Nejv\u00FDznamn\u011Bj\u0161\u00ED \u00FAlohu v aktivaci basofil\u016F a \u017E\u00EDrn\u00FDch bun\u011Bk sehr\u00E1v\u00E1 vysokoafinitn\u00ED Fc\u03B5RI receptor. Jeho p\u0159emost\u011Bn\u00ED IgE molekulami je sign\u00E1lem pro zah\u00E1jen\u00ED aktiva\u010Dn\u00ED kask\u00E1dy \u017E\u00EDrn\u00FDch bun\u011Bk a basofil\u016F, vedouc\u00ED k uvoln\u011Bn\u00ED medi\u00E1tor\u016F alergick\u00E9ho z\u00E1n\u011Btu. Mezi hlavn\u00ED tyrosinkin\u00E1zy pod\u00EDlej\u00EDc\u00ED se na Fc\u03B5RI mediovan\u00FDch signaliza\u010Dn\u00EDch drah\u00E1ch pat\u0159\u00ED proteinov\u00E9 tyrosinkin\u00E1zy Fyn, Lyn a Syk vedouc\u00ED k fosforylaci molekul sign\u00E1ln\u00EDch drah. Diagnostika alergie u pacient\u016F s hypo-, resp. agamaglobulin\u00E9mi\u00ED je zna\u010Dn\u011B problematick\u00E1 vzhledem k n\u00EDzk\u00E9 koncentraci nebo absenci IgE. I kdy\u017E se zd\u00E1, \u017Ee vznik atopie je u t\u011Bchto pacient\u016F zna\u010Dn\u011B spekulativn\u00ED , \u0159ada z nich vykazuje symptomy charakteristick\u00E9 pro alergickou hypersenzitivitu. Je zn\u00E1mo, \u017Ee \u0159ada imunodeficit\u016F je spojena rovn\u011B\u017E s defekty ur\u010Dit\u00FDch sign\u00E1ln\u00EDch drah. V na\u0161\u00ED pr\u00E1ci jsme zji\u0161\u0165ovali, zda status non-respond\u00E9ra v p\u0159\u00EDpad\u011B pacient\u016F s hypo-, resp. agamaglobulin\u00E9mi\u00ED je spojen pouze s absenc\u00ED IgE, nebo jsou p\u0159\u00EDtomny i zm\u011Bny ve funkci p\u0159\u00EDslu\u0161n\u00FDch sign\u00E1ln\u00EDch drah. Uk\u00E1zalo se, \u017Ee protein\u00E1za Btk (Bruton tyrosinkin\u00E1za) hraje rozhoduj\u00EDc\u00ED \u00FAlohu nejen v p\u0159\u00EDpad\u011B B-lymfocyt\u016F, ale rovn\u011B\u017E v p\u0159\u00EDpad\u011B bun\u011B\u010Dn\u00E9 aktivace mediovan\u00E9 Fc\u03B5RI."@cs . . . . "14" . "[52D4C5892D0B]" . . . "Basofily a \u017E\u00EDrn\u00E9 bu\u0148ky p\u016Fsob\u00ED jako regula\u010Dn\u00ED a efektorov\u00E9 bu\u0148ky p\u0159irozen\u00FDch imunitn\u00EDch mechanism\u016F a v sou\u010Dasn\u00E9 dob\u011B jsou pova\u017Eov\u00E1ny za spojnici mezi vrozenou a adaptivn\u00ED imunitou. Spolu s \u017E\u00EDrn\u00FDmi bu\u0148kami se pod\u00EDlej\u00ED na vzniku alergick\u00E9 reakce okam\u017Eit\u00E9ho typu zprost\u0159edkovan\u00E9 IgE. Nejv\u00FDznamn\u011Bj\u0161\u00ED \u00FAlohu v aktivaci basofil\u016F a \u017E\u00EDrn\u00FDch bun\u011Bk sehr\u00E1v\u00E1 vysokoafinitn\u00ED Fc\u03B5RI receptor. Jeho p\u0159emost\u011Bn\u00ED IgE molekulami je sign\u00E1lem pro zah\u00E1jen\u00ED aktiva\u010Dn\u00ED kask\u00E1dy \u017E\u00EDrn\u00FDch bun\u011Bk a basofil\u016F, vedouc\u00ED k uvoln\u011Bn\u00ED medi\u00E1tor\u016F alergick\u00E9ho z\u00E1n\u011Btu. Mezi hlavn\u00ED tyrosinkin\u00E1zy pod\u00EDlej\u00EDc\u00ED se na Fc\u03B5RI mediovan\u00FDch signaliza\u010Dn\u00EDch drah\u00E1ch pat\u0159\u00ED proteinov\u00E9 tyrosinkin\u00E1zy Fyn, Lyn a Syk vedouc\u00ED k fosforylaci molekul sign\u00E1ln\u00EDch drah. Diagnostika alergie u pacient\u016F s hypo-, resp. agamaglobulin\u00E9mi\u00ED je zna\u010Dn\u011B problematick\u00E1 vzhledem k n\u00EDzk\u00E9 koncentraci nebo absenci IgE. I kdy\u017E se zd\u00E1, \u017Ee vznik atopie je u t\u011Bchto pacient\u016F zna\u010Dn\u011B spekulativn\u00ED , \u0159ada z nich vykazuje symptomy charakteristick\u00E9 pro alergickou hypersenzitivitu. Je zn\u00E1mo, \u017Ee \u0159ada imunodeficit\u016F je spojena rovn\u011B\u017E s defekty ur\u010Dit\u00FDch sign\u00E1ln\u00EDch drah. V na\u0161\u00ED pr\u00E1ci jsme zji\u0161\u0165ovali, zda status non-respond\u00E9ra v p\u0159\u00EDpad\u011B pacient\u016F s hypo-, resp. agamaglobulin\u00E9mi\u00ED je spojen pouze s absenc\u00ED IgE, nebo jsou p\u0159\u00EDtomny i zm\u011Bny ve funkci p\u0159\u00EDslu\u0161n\u00FDch sign\u00E1ln\u00EDch drah. Uk\u00E1zalo se, \u017Ee protein\u00E1za Btk (Bruton tyrosinkin\u00E1za) hraje rozhoduj\u00EDc\u00ED \u00FAlohu nejen v p\u0159\u00EDpad\u011B B-lymfocyt\u016F, ale rovn\u011B\u017E v p\u0159\u00EDpad\u011B bun\u011B\u010Dn\u00E9 aktivace mediovan\u00E9 Fc\u03B5RI." . . . . "Nov\u00E1k, V." . "Lochmanov\u00E1, A." . "Funk\u010Dn\u00ED aktivita Fc\u03B5RI receptoru u osob s protil\u00E1tkov\u00FDmi deficity"@cs . . "Functional activity of Fc\u03B5RI receptor in patients with immunoglobulin deficiency"@en . "basophils; allergy; IgE; Fc\u03B5RI receptor; Bruton tyrosinkinase"@en . . . . . "Funk\u010Dn\u00ED aktivita Fc\u03B5RI receptoru u osob s protil\u00E1tkov\u00FDmi deficity"@cs . "3" . "Funk\u010Dn\u00ED aktivita Fc\u03B5RI receptoru u osob s protil\u00E1tkov\u00FDmi deficity" . "3"^^ . "Alergie" . "Basophils and mast cells act as regulatory and effector cells of natural immune mechanisms and are currently considered as a bridge between innate and adaptive immunity. Along with mast cells they play pivotal roles in the initiation of allergic reaction. IgE cross-linking of the highaffinity Fc\u03B5RI receptor on these cells activates intracellular signaling pathways that led to the degranulation and release of allergic inflammaton mediators. The main tyrosine kinase involved in signaling pathwas mediated Fc\u03B5RI include protein tyrosine kinase Fyn, Lyn and Syk, leading to phosphorylation of signaling pathways. Patients with immunoglobulin deficiency do not react to positive, e.g. anti-IgE stimulation because of lack of IgE. It is generally known that immunedeficiencies are connected with defects in signal transduction pathways. The aim of our study was to find out whether the non-responder status in patients with hypo-, resp. agamaglobulinemia is related only to the absence of IgE or whether some dysfunctions in basophil signaling are also present. Our results confirmed that BTK plays crucial role in B-lymphocytes as well as mast cell activation through Fc\u03B5RI."@en . "Functional activity of Fc\u03B5RI receptor in patients with immunoglobulin deficiency"@en . "1212-3536" . . "V" . "1"^^ . "Funk\u010Dn\u00ED aktivita Fc\u03B5RI receptoru u osob s protil\u00E1tkov\u00FDmi deficity" . "\u0160kopkov\u00E1, Olga" . "4"^^ . . "RIV/00843989:_____/12:00102611" . . . "137425" . "CZ - \u010Cesk\u00E1 republika" .