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C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation but also as the major regulator of the contact system. C1-inhibitor is an acute-phase protein that circulates in blood at levels of around 0.25 g/L. The levels rise ~2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated. This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named after its complement inhibitory activity, C1-inhibitor also i

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  • مثبط سي1 (ar)
  • C1-Esterase-Inhibitor (de)
  • C1-inhibitor (en)
  • Inhibidor C1 (es)
  • C1-Inhibiteur (fr)
  • C1-inibitore (it)
  • Inhibitor C1 (pl)
  • SERPING1 (uk)
rdfs:comment
  • مثبط سي1 (بالإنجليزية: C1-inhibitor)‏ هو بروتين مانع أو مثبط ينتمي إلى فصيلة بروتينات السربين. وظيفته الرئيسية هي تثبيط النظام مكملًا لمنع تفعيل عفوية. يؤدي خلل هذا البروتين إلى إنتاج كمية قليلة، أو معيوبة، من البروتين. (ar)
  • Der C1-Esterase-Inhibitor (C1-INH, C1-Inhibitor) ist ein zu den Serpinen gehörender Serin-Protease-Inhibitor, der die Aktivierung des Komplementfaktors C1 kontrolliert und so das Komplementsystem reguliert. Der Normwert im Blut beträgt etwa 0,25–0,45 g/l. (de)
  • El inhibidor C1 (INH C1) es una proteína inhibidora de la proteasa serina (serpina), cuya principal función consiste en inhibir el sistema complemento. Circula en la sangre a niveles que oscilan entre 0,25 y 0,45 g/l. (es)
  • C1-inhibiteur (C1inh) ou inhibiteur de la C1 estérase est un inhibiteur de sérine protéase (serpine), dont la principale fonction est d’inhiber le système du complément. Il circule dans le sang à des concentrations de 0,25-0,45 g/l environ. (fr)
  • C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation but also as the major regulator of the contact system. C1-inhibitor is an acute-phase protein that circulates in blood at levels of around 0.25 g/L. The levels rise ~2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated. This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named after its complement inhibitory activity, C1-inhibitor also i (en)
  • Il C1 inibitore (C1-INH) è una proteina (serpina), la cui funzione principale è l'inibizione del sistema del complemento. Questa proteina circola nel sangue a livelli di circa 0,25-0,45 g/l. I livelli aumentano di circa 2 volte durante l'infiammazione. L'inibitore C1 si lega irreversibilmente e inattiva le proteasi C1r e C1s nel complesso C1 della via classica del complemento. Anche le proteasi MASP-1 e MASP-2 nei complessi MBL della via della lectina sono inattivate. In questo modo, l'inibitore C1 impedisce la scissione proteolitica dei componenti successivi del complemento C4 e C2 da parte di C1 e MBL. Anche se prende il nome dalla sua attività inibitoria del complemento, l'inibitore C1 inibisce anche le proteasi delle vie fibrinolitiche, della coagulazione e della chinina. Si noti che (it)
  • Inhibitor C1 (ang. C1 inhibitor, C1-inh, C1 esterase inhibitor) – należący do serpin, białkowy inhibitor proteaz serynowych, którego jedną z głównych funkcji jest przeciwdziałanie spontanicznej aktywacji dopełniacza. Inhibitor C1 jest białkiem ostrej fazy, α2-globuliną, która jest prawidłowym składnikiem białek osocza – występuje w stężeniu 0,25-0,45 g/l. Stężenie to ulega 2-3-krotnemu zwiększeniu w przebiegu reakcji zapalnej. Jest produkowany w wątrobie, ale także przez pobudzone monocyty i inne komórki. Ludzki gen dla inhibitora C1 (SERPING1) znajduje się na 11 chromosomie (11q11-q13.1) (pl)
  • SERPING1 (англ. Serpin family G member 1) – білок, який кодується однойменним геном, розташованим у людей на короткому плечі 11-ї хромосоми. Довжина поліпептидного ланцюга білка становить 500 амінокислот, а молекулярна маса — 55 154. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин (uk)
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