| Nome: | Descrição: | Tamanho: | Formato: | |
|---|---|---|---|---|
| 263.39 KB | Adobe PDF |
Orientador(es)
Resumo(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.
Descrição
Palavras-chave
Paroxysmal nocturnal hemoglobinuria PIG-A gene Clonal expansion
Contexto Educativo
Citação
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
Editora
Escola Superior de Saúde P.Porto