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Article

Complement- and inflammasome-mediated autoinflammation-paroxysmal nocturnal hemoglobinuria

2019-05-13

Abstract excerpt

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder characterized by complement-mediated hemolysis and thrombosis, and bone marrow failure. Affected cells harbor somatic mutation in X-linked PIGA gene, essential for the initial step in glycosylphosphatidylinositol (GPI) biosynthesis. Loss of GPI biosynthesis results in defective cell-surface expression of GPI-anchored complem...

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Literature Corpus work
34ed9acf-85d0-5fd8-991b-d9df3d3d894f
DOI
10.1101/635573
Open publication

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