نتایج جستجو برای: complement c1 inhibitor proteins

تعداد نتایج: 779838  

Journal: :The Journal of clinical investigation 1989
J H Nuijens A J Eerenberg-Belmer C C Huijbregts W O Schreuder R J Felt-Bersma J J Abbink L G Thijs C E Hack

Activation of both the complement system and the contact system of intrinsic coagulation is implicated in the pathophysiology of sepsis. Because C1 inhibitor (C1-Inh) regulates the activation of both cascade systems, we studied the characteristics of plasma C1-Inh in 48 patients with severe sepsis on admission to the Intensive Care Unit at the Free University of Amsterdam. The ratio between the...

2015
Silke Wissing Jens Wölfel Helmut Kewes Christian Niehus Corinna Bialek Sabine Hertel Nicole Faust

Background Due to their clinical importance, the development of therapeutic proteins has accelerated immensely over the past years. However, the expression of highly glycosylated recombinant therapeutic proteins, as for example blood coagulation factors or serum proteins has remained a challenging task. Human cell lines, as HEK293 cells or our amniocyte-derived CAP cell line, appear to be effic...

Journal: :Journal of clinical pathology 2004
Y Karim H Griffiths S Deacock

This report describes a patient with hereditary angioedema (HAE) in whom complement C4 values were consistently normal. There was a family history of HAE, for which the patient had previously been screened, but in view of her normal C4 values she was deemed unaffected. However, at 10 years of age she presented with an eight month history of episodes of swelling affecting her hands and recurrent...

Journal: :International immunology 1998
M H Nissen S Bregenholt J A Nording M H Claesson

We have previously shown that activated C1s complement and activated T cells cleave beta2-microglobulin (beta2m) in vitro leading to the formation of desLys58 beta2m. This process can specifically be inhibited by C1-esterase inhibitor (C1-inh). Furthermore we showed that exogenously added desLys58 beta2m in nanomolar amounts to a one-way allogenic mixed lymphocyte culture (MLC) increased the en...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2013
Umakhanth Venkatraman Girija Alexandre R Gingras Jamie E Marshall Roshni Panchal Md Arif Sheikh James A J Harper Péter Gál Wilhelm J Schwaeble Daniel A Mitchell Peter C E Moody Russell Wallis

Complement component C1, the complex that initiates the classical pathway of complement activation, is a 790-kDa assembly formed from the target-recognition subcomponent C1q and the modular proteases C1r and C1s. The proteases are elongated tetramers that become more compact when they bind to the collagen-like domains of C1q. Here, we describe a series of structures that reveal how the subcompo...

2018
Pamela S. Hair Adrianne I. Enos Neel K. Krishna Kenji M. Cunnion

Two major aspects of systemic lupus erythematosus (SLE) pathogenesis that have yet to be targeted therapeutically are immune complex-initiated complement activation and neutrophil extracellular trap (NET) formation by neutrophils. Here, we report in vitro testing of peptide inhibitor of complement C1 (PIC1) in assays of immune complexmediated complement activation in human sera and assays for N...

Abolfazl Barzegar Bafrouei, Elham karimipour, Moosa Javdani,

A part of the immune system that improves (complements) the ability of antibodies and phagocytic cells to clear microorganisms and injured cells from an organism, attacks the pathogen's cell membrane, and encourages inflammation called complement system. It is main part of immune system. Over thirty proteins and protein pieces compose the complement system, including cell membrane receptors, an...

Journal: :The American journal of pathology 2010
Giuseppe Castellano Rita Melchiorre Antonia Loverre Pasquale Ditonno Vincenzo Montinaro Michele Rossini Chiara Divella Michele Battaglia Giuseppe Lucarelli Gennaro Annunziata Silvano Palazzo Francesco Paolo Selvaggi Francesco Staffieri Antonio Crovace Mohamed R Daha Maurice Mannesse Sandra van Wetering Francesco Paolo Schena Giuseppe Grandaliano

Ischemia-reperfusion injury is the major cause of delayed graft function in transplanted kidneys, an early event significantly affecting long-term graft function and survival. Several studies in rodents suggest that the alternative pathway of the complement system plays a pivotal role in renal ischemia-reperfusion injury. However, limited information is currently available from humans and large...

Journal: :Chest 2021

TOPIC: Allergy and Airway TYPE: Medical Student/Resident Case Reports INTRODUCTION: The pathophysiology of angioedema is broad. It may be hereditary, idiopathic, or a reaction to range medications allergens; there have been few case reports describing recurrent in patients with autoimmune conditions such as systemic lupus erythematosus (SLE). We present the patient SLE who suffered from face, i...

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