Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion

Peixoto, Vanda; Vieira da Silva, Manuela; Prudêncio, Cristina

http://hdl.handle.net/10400.22/25679

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Authors

Peixoto, Vanda

Vieira da Silva, Manuela

Prudêncio, Cristina

Abstract(s)

Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare, acquired clonal disease of bone marrow stem-cells, genetically characterized by the somatic mutation of the phosphatidylinositol glycan class A (PIG-A) gene. That leads to defective synthesis of glycosylphosphatidylinositol (GPI) responsible for anchorage and fixation of surface proteins like complement decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59). These proteins protect red blood cells from lysis by activated complement, leading to intravascular hemolysis.

Keywords

Paroxysmal nocturnal hemoglobinuria PIG-A gene Clonal expansion

URI

http://hdl.handle.net/10400.22/25679

Citation

Peixoto, V., Vieira, M., & Prudêncio, C. (2018). Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion. Book of abstracts of the III Encontro de Biotecnologia Medicinal/ I Iberian Congress on Medicinal Biotechnology, 48. https://paginas.ess.ipp.pt/ebtm/2018/3EBtM_BookOfAbstracts.pdf