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Repositório Científico do Instituto Politécnico do Porto
Conference object . 2018
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Data sources: Repositório Científico do Instituto Politécnico do Porto
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Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion

descriptionPublicationkeyboard_double_arrow_right Conference object 18 May 2018 Portugal English Publisher:Escola Superior de Saúde P.Porto
Authors: Peixoto, Vanda; Vieira da Silva, Manuela; Prudêncio, Cristina;

handle: 10400.22/25679

Mechanisms of paroxysmal nocturnal hemoglobinuria clonal expansion

Abstract

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.

Country
Portugal
Related Organizations
Keywords

PIG-A gene, Paroxysmal nocturnal hemoglobinuria, Clonal expansion

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
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