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13 mar 2023 · The most common type of primary immunodeficiency is antibody deficiency consisting of approximately 65% of the cases. In the cases of meningococcal infection, the prevalence rate is nearly 30%. Patients with C1q deficiency are observed to have a 93% chance of having SLE in the future.
17 lut 2020 · Patients with C1, C2 or C4 deficiency have an increased occurrence of autoantibodies; antinuclear antibodies are present in 75% of patients with C1 or C4 deficiency and 25–55% of patients with C2 deficiency.
Antibody-mediated complement activation leads to MAC deposition on the surface of infected cells. [1] Assembly of the MAC leads to pores that disrupt the cell membrane of target cells, leading to cell lysis and death.
Antibody-mediated complement activation leads to MAC deposition on the surface of IFN-γ–primed human ECs. MAC is internalized by a clathrin-mediated endocytosis and delivered to Ras-related protein Rab5 + endosomes.
An overview of membrane attack complex deficiency, including C5 deficiency, C6 deficiency, C7 deficiency, C8 deficiency and C9 deficiency.
1 cze 2020 · Antibody-mediated complement activation leads to MAC deposition on the surface of IFN-γ–primed human ECs. MAC is internalized by a clathrin-mediated endocytosis and delivered to Ras-related protein Rab5 + endosomes.
Unlike C6SD or C6SD/C7SD, C7SD or C9SD will, due to C5b6- or C5b8-triggered consumption, both phenotypically and clinically behave like a complete deficiency, with a likelihood of meningococcal disease (similarly, it cannot be fully excluded that the present C7 case is actually subtotal).