Matches in SemOpenAlex for { <https://semopenalex.org/work/W190030527> ?p ?o ?g. }
- W190030527 endingPage "137" @default.
- W190030527 startingPage "113" @default.
- W190030527 abstract "Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder in which PIG-A mutant clones of hematopoietic stem cells undergo massive but nonneoplastic expansion. The only known consequence of PIG-A gene dysfunction is the loss of glycosylphosphatidylinositol anchored proteins (GPI-AP) expression by the mutant hematopoietic stem cells and its progeny. In erythrocytes, this defect results in episodes of intravascular hemolysis when complement is activated in the context of inflammation; in platelets, the defect is believed to favor thrombus formation. On the other hand, PIG-A mutations do not appear to directly give rise to the bone marrow aplasia that frequently accompanies PNH. Instead, it would seem that the underlying pathophysiologic process of aplastic anemia confers a relative survival advantage to PIG-A mutant stem cells. Clarifying the molecular basis for this “unnatural” selection process will likely impact the understanding not only of PNH, AA, and other autoimmune syndromes, but also of the unique biology of GPI-AP as well as the adaptive physiology of hematopoietic stem cells." @default.
- W190030527 created "2016-06-24" @default.
- W190030527 creator A5015033246 @default.
- W190030527 creator A5060331249 @default.
- W190030527 creator A5086021239 @default.
- W190030527 date "2000-01-01" @default.
- W190030527 modified "2023-09-28" @default.
- W190030527 title "Bone Marrow Failure in PNH" @default.
- W190030527 cites W105676805 @default.
- W190030527 cites W123419685 @default.
- W190030527 cites W146246930 @default.
- W190030527 cites W1494325974 @default.
- W190030527 cites W1508015584 @default.
- W190030527 cites W1510857662 @default.
- W190030527 cites W1534165148 @default.
- W190030527 cites W156415080 @default.
- W190030527 cites W1578346366 @default.
- W190030527 cites W1606956077 @default.
- W190030527 cites W1608329801 @default.
- W190030527 cites W16534552 @default.
- W190030527 cites W1957167758 @default.
- W190030527 cites W1965604791 @default.
- W190030527 cites W1968736929 @default.
- W190030527 cites W1969158591 @default.
- W190030527 cites W1969466880 @default.
- W190030527 cites W1973311214 @default.
- W190030527 cites W1973606894 @default.
- W190030527 cites W1974559436 @default.
- W190030527 cites W1978373343 @default.
- W190030527 cites W1978675332 @default.
- W190030527 cites W1980466505 @default.
- W190030527 cites W1982133573 @default.
- W190030527 cites W1982381511 @default.
- W190030527 cites W1984468406 @default.
- W190030527 cites W1988090285 @default.
- W190030527 cites W1990050859 @default.
- W190030527 cites W1991319226 @default.
- W190030527 cites W1992685946 @default.
- W190030527 cites W1994940934 @default.
- W190030527 cites W2002927121 @default.
- W190030527 cites W2003593894 @default.
- W190030527 cites W2005157565 @default.
- W190030527 cites W2005327191 @default.
- W190030527 cites W2005978032 @default.
- W190030527 cites W2007552143 @default.
- W190030527 cites W2007840325 @default.
- W190030527 cites W2008139210 @default.
- W190030527 cites W2008177711 @default.
- W190030527 cites W2009387411 @default.
- W190030527 cites W2013641123 @default.
- W190030527 cites W2014078232 @default.
- W190030527 cites W2015586987 @default.
- W190030527 cites W2021789061 @default.
- W190030527 cites W2021918217 @default.
- W190030527 cites W2022589671 @default.
- W190030527 cites W2025123869 @default.
- W190030527 cites W2028093783 @default.
- W190030527 cites W2029113059 @default.
- W190030527 cites W2029549314 @default.
- W190030527 cites W2032463909 @default.
- W190030527 cites W2032654435 @default.
- W190030527 cites W2032838364 @default.
- W190030527 cites W2040728773 @default.
- W190030527 cites W2041490149 @default.
- W190030527 cites W2042038557 @default.
- W190030527 cites W2053432732 @default.
- W190030527 cites W2054323447 @default.
- W190030527 cites W205703609 @default.
- W190030527 cites W2064539486 @default.
- W190030527 cites W2069757957 @default.
- W190030527 cites W2071354240 @default.
- W190030527 cites W2072926018 @default.
- W190030527 cites W2073925818 @default.
- W190030527 cites W2077628369 @default.
- W190030527 cites W2077885787 @default.
- W190030527 cites W2081978633 @default.
- W190030527 cites W2083031926 @default.
- W190030527 cites W2089001511 @default.
- W190030527 cites W2089476060 @default.
- W190030527 cites W2095335458 @default.
- W190030527 cites W2123150576 @default.
- W190030527 cites W2126544407 @default.
- W190030527 cites W2130487816 @default.
- W190030527 cites W2132065088 @default.
- W190030527 cites W2133308882 @default.
- W190030527 cites W2137579630 @default.
- W190030527 cites W2139689892 @default.
- W190030527 cites W2144273224 @default.
- W190030527 cites W2148240710 @default.
- W190030527 cites W2155462087 @default.
- W190030527 cites W2160942082 @default.
- W190030527 cites W2164339702 @default.
- W190030527 cites W2164711235 @default.
- W190030527 cites W2165325928 @default.
- W190030527 cites W2166670696 @default.
- W190030527 cites W2168159922 @default.
- W190030527 cites W2168725807 @default.
- W190030527 cites W2198426544 @default.