p(HGNC:CD59)
The endothelium normally expresses high levels of CD59, which protects endothelial cells in healthy individuals and in PNH patients from MAC-mediated injury. PubMed:29929138
Interestingly, we have recently reported an association with venous thrombosis for lack of CD55 [45], so PNH patients lacking both CD55 and CD59 may suffer from both venous and arterial thrombosis. PubMed:29929138
CD59 deficiency is a common finding in adult patients with PNH, which is characterized by clonal expansion of hematopoietic stem cells that have acquired a mutation in the PIGA gene (phosphatidylinositol glycan anchor biosynthesis, class A). PubMed:29929138
Interestingly, we have recently reported an association with venous thrombosis for lack of CD55 [45], so PNH patients lacking both CD55 and CD59 may suffer from both venous and arterial thrombosis. PubMed:29929138
PIGA encodes a GPI biosynthesis protein, phosphatidylinositol N-acetylglucosaminyltransferase subunit A [5, 6], and erythrocytes deficient in GPI-anchored membrane proteins, including CD59, undergo complement-mediated hemolysis. PubMed:29929138
This scenario may resemble that of paroxysmal nocturnal hemoglobinuria patients, who can be treated with complement C5- blocking antibody eculizumab [80]; in this example, extravascular hemolysis has been shown to occur in a small number of these patients despite treatment. It has been suggested that this is due to erythrophagocytosis of red blood cells deficient in the C3d-opsonized complement regulators – CD55 and CD59 [81,82]. PubMed:26875449
PIGA encodes a GPI biosynthesis protein, phosphatidylinositol N-acetylglucosaminyltransferase subunit A [5, 6], and erythrocytes deficient in GPI-anchored membrane proteins, including CD59, undergo complement-mediated hemolysis. PubMed:29929138
CD59 deficiency is a common finding in adult patients with PNH, which is characterized by clonal expansion of hematopoietic stem cells that have acquired a mutation in the PIGA gene (phosphatidylinositol glycan anchor biosynthesis, class A). PubMed:29929138
The endothelium normally expresses high levels of CD59, which protects endothelial cells in healthy individuals and in PNH patients from MAC-mediated injury. PubMed:29929138
Interestingly, we have recently reported an association with venous thrombosis for lack of CD55 [45], so PNH patients lacking both CD55 and CD59 may suffer from both venous and arterial thrombosis. PubMed:29929138
Interestingly, we have recently reported an association with venous thrombosis for lack of CD55 [45], so PNH patients lacking both CD55 and CD59 may suffer from both venous and arterial thrombosis. PubMed:29929138
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If you find BEL Commons useful in your work, please consider citing: Hoyt, C. T., Domingo-Fernández, D., & Hofmann-Apitius, M. (2018). BEL Commons: an environment for exploration and analysis of networks encoded in Biological Expression Language. Database, 2018(3), 1–11.