![]() 1, 2 There are 3 distinct pathways through which the complement cascade can be activated on different molecules for their initiation (classical, lectin, and alternative), but these pathways converge at the point of C3 in the cascade to generate the same set of effector molecules. Opsonization is the process of marking pathogens for destruction by recognizing and targeting invading particles for phagocytosis by coating them with antibodies or complement components. The complement system is part of the innate immune response system, composed of more than 50 distinct plasma and serum proteins that interact to opsonize pathogens and induce inflammatory responses to fight infection. The complement system plays an important role in mediating the hemolysis that characterizes paroxysmal nocturnal hemoglobinuria (PNH). Shammo consults for Apellis Pharmaceuticals. Sarda is an employee of Apellis Pharmaceuticals. Bektas, Copley-Merriman, and Khan are employees of RTI Health Solutions. ![]() If left untreated, PNH has a 10-year mortality rate of 29%, although the natural history of this disease has been recently altered by the introduction of complement inhibitors for the treatment of PNH.ĭISCLOSURES: This research was developed under a research contract between RTI Health Solutions and Apellis Pharmaceuticals and was funded by Apellis Pharmaceuticals. Epidemiologic data, while sparse, suggest that an estimated 5,000-6,000 individuals in the United States are affected by PNH. PNH is characterized by fatigue hemolytic anemia that can be severe and debilitating increased lactic dehydrogenase level, reticulocyte count, and bilirubin level propensity for thrombotic events and renal dysfunction. The complement system destroys cells without these protective proteins, resulting in general hemolysis. These stem cells produce abnormal clone blood cells that lack the complement regulatory proteins CD55 and CD59, causing the body to recognize these otherwise healthy red blood cells as damaged. ![]() ![]() Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, chronic, acquired, hematologic disease caused by somatic mutations in the gene PIGA in the hematopoietic stem cells. The role of the complement system is 2-fold: immune surveillance and host defense. The complement system is part of the innate immune response system, which comprises more than 50 distinct plasma and serum proteins that interact to opsonize pathogens (i.e., mark pathogens for destruction) and induce inflammatory responses to fight infection. ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |