نتایج جستجو برای: primary c3 deficiency

تعداد نتایج: 784306  

Journal: :The Journal of Experimental Medicine 1998
Craig Hammerberg Santosh K. Katiyar Michael C. Carroll Kevin D. Cooper

Complement component 3 (C3), a critical regulator of innate immunity, may also play a role in the regulation of cognate immunity, such as contact sensitivity responses. Because ultraviolet (UV) radiation also activates C3 in the skin, we determined whether the immunosuppressed state that results when a contact sensitizer is applied through UVB-exposed skin requires the presence and activation o...

2013
Nilhan Rajiva de Silva Sepali Gunawardena Damayanthi Rathnayake Geethani Devika Wickramasingha

BACKGROUND While primary immunodeficiencies (PID has been recognized in the west for decades, recognition has been delayed in the third world. This study attempts to detail the spectrum of PID, the therapy provided, and constraints in the diagnosis and treatment in a middle income country such as Sri Lanka. METHODS Nine hundred and forty two patients with recurrent infections and features sug...

2013
E. J. ter Borg H. van Velzen-Blad D. Hamann

A lupus patient with a clinically quiescent disease stage will be described who had severely depressed C4 levels while levels of C3 en CH50 were normal. Additional testing revealed a homozygous C4A isotype deficiency as the cause of the very low C4 levels. It should be emphasized that in SLE, a (very) low C4 level does not always means (subclinical) disease activity.

Journal: :Nephrology Dialysis Transplantation 2023

Abstract Background and Aims Certain thrombotic microangiopathies (TMA) respond to inhibition of complement factor C5, which abrogates formation the membrane attack complex hence impairs host defense. Inhibition C5a receptor (C5aR) avoids this safety problem still controls autoimmune systemic vasculitis. We speculated that targeting C5aR could be sufficient also in TMA, e.g., related cholestero...

Journal: :Blood 2009
Antonio M Risitano Rosario Notaro Ludovica Marando Bianca Serio Danilo Ranaldi Elisa Seneca Patrizia Ricci Fiorella Alfinito Andrea Camera Giacomo Gianfaldoni Angela Amendola Carla Boschetti Eros Di Bona Giorgio Fratellanza Filippo Barbano Francesco Rodeghiero Alberto Zanella Anna Paola Iori Carmine Selleri Lucio Luzzatto Bruno Rotoli

In paroxysmal nocturnal hemoglobinuria (PNH) hemolytic anemia is due mainly to deficiency of the complement regulator CD59 on the surface of red blood cells (RBCs). Eculizumab, an antibody that targets complement fraction 5 (C5), has proven highly effective in abolishing complement-mediated intravascular hemolysis in PNH; however, the hematologic benefit varies considerably among patients. In t...

Journal: :The Journal of Experimental Medicine 1971
Michael M. Frank Joseph May Thelma Gaither Leonard Ellman

In vitro studies were performed utilizing sera from a strain of guinea pigs with a total absence of hemolytically active C4. Previous studies in these animals have demonstrated normal complement-dependent inflammatory reactions, suggesting that they are able to bypass their deficiency of C4. In vitro studies with C4-deficient serum also indicate normal activation of late-acting C components. Th...

Journal: :Gut 1973
B J Potter A M Trueman E A Jones

Total serum haemolytic complement activity (CH(50)) and the serum concentrations of both the third and fourth components of the complement system (C3 and C4) have been measured in 29 control subjects, 92 patients with chronic hepatocellular disease, and eight patients with large duct biliary tract obstruction. The mean C4 concentration was reduced in all types of chronic liver disease studied. ...

Journal: :Journal of clinical pathology 1991
E T Littleton L Bevis L J Hansen M Peakman A P Mowat G Mieli-Vergani D Vergani

Activation of the complement system, the main humoral mediator of inflammation, is restrained by the action of enzyme inhibitors including alpha 1-antitrypsin. Deficiency leads to chronic liver disease in about one in five children with this genetic defect. Complement activation was investigated in 34 children with alpha 1 AT deficiency (12 with minimal, 10 with moderate, and 12 with severe liv...

Journal: :The Journal of veterinary medical science 2000
Y Watanabe M Morimatsu B Syuto

Changes in the lactate dehydrogenase (LDH) isozyme pattern of primary culture of neurons treated with botulinum C3 enzyme were examined in order to elucidate the functional changes accompanying the morphological change that follows ADP-ribosylation of Rho protein. Primary neurons were prepared from the cerebrum of ICR mouse embryos on day 15. Neurons were cultured in MEM with 10% fetal calf ser...

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