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- W2017183118 abstract "Abstract Atypical hemolytic uremic syndrome (aHUS) may be associated with mutations in the C-terminal of factor H (FH). FH binds to platelets via the C-terminal as previously shown using a construct consisting of short consensus repeats (SCRs) 15 to 20. A total of 4 FH mutations, in SCR15 (C870R) and SCR20 (V1168E, E1198K, and E1198Stop) in patients with aHUS, were studied regarding their ability to allow complement activation on platelet surfaces. Purified FH-E1198Stop mutant exhibited reduced binding to normal washed platelets compared with normal FH, detected by flow cytometry. Washed platelets taken from the 4 patients with aHUS during remission exhibited C3 and C9 deposition, as well as CD40-ligand (CD40L) expression indicating platelet activation. Combining patient serum/plasma with normal washed platelets led to C3 and C9 deposition, CD40L and CD62P expression, aggregate formation, and generation of tissue factor-expressing microparticles. Complement deposition and platelet activation were reduced when normal FH was preincubated with platelets and were minimal when using normal serum. The purified FH-E1198Stop mutant added to FH-deficient plasma (complemented with C3) allowed considerable C3 deposition on washed platelets, in comparison to normal FH. In summary, mutated FH enables complement activation on the surface of platelets and their activation, which may contribute to the development of thrombocytopenia in aHUS." @default.
- W2017183118 created "2016-06-24" @default.
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- W2017183118 date "2008-06-01" @default.
- W2017183118 modified "2023-10-03" @default.
- W2017183118 title "Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation" @default.
- W2017183118 cites W111332543 @default.
- W2017183118 cites W1507314459 @default.
- W2017183118 cites W1529745308 @default.
- W2017183118 cites W1561516029 @default.
- W2017183118 cites W1570017296 @default.
- W2017183118 cites W1595344282 @default.
- W2017183118 cites W165853039 @default.
- W2017183118 cites W1758130036 @default.
- W2017183118 cites W1824094556 @default.
- W2017183118 cites W1927547718 @default.
- W2017183118 cites W1965972308 @default.
- W2017183118 cites W1966590389 @default.
- W2017183118 cites W1970738865 @default.
- W2017183118 cites W1974010028 @default.
- W2017183118 cites W1979111981 @default.
- W2017183118 cites W1986066232 @default.
- W2017183118 cites W1990529812 @default.
- W2017183118 cites W2011323677 @default.
- W2017183118 cites W2014670467 @default.
- W2017183118 cites W2021455198 @default.
- W2017183118 cites W2030665169 @default.
- W2017183118 cites W2031667319 @default.
- W2017183118 cites W2036566635 @default.
- W2017183118 cites W2043018978 @default.
- W2017183118 cites W2048368670 @default.
- W2017183118 cites W2054576721 @default.
- W2017183118 cites W2054774042 @default.
- W2017183118 cites W205927857 @default.
- W2017183118 cites W2068933681 @default.
- W2017183118 cites W2069015356 @default.
- W2017183118 cites W2072417552 @default.
- W2017183118 cites W2075013504 @default.
- W2017183118 cites W207713798 @default.
- W2017183118 cites W2079104693 @default.
- W2017183118 cites W2081978633 @default.
- W2017183118 cites W2087564804 @default.
- W2017183118 cites W2093493827 @default.
- W2017183118 cites W2095793217 @default.
- W2017183118 cites W2098315002 @default.
- W2017183118 cites W2098383030 @default.
- W2017183118 cites W2098847286 @default.
- W2017183118 cites W2100963035 @default.
- W2017183118 cites W2102898694 @default.
- W2017183118 cites W2103769076 @default.
- W2017183118 cites W2106578061 @default.
- W2017183118 cites W2113053099 @default.
- W2017183118 cites W2114738843 @default.
- W2017183118 cites W2117066664 @default.
- W2017183118 cites W2124841845 @default.
- W2017183118 cites W2124946560 @default.
- W2017183118 cites W2128624830 @default.
- W2017183118 cites W2130327650 @default.
- W2017183118 cites W2131509889 @default.
- W2017183118 cites W2134898804 @default.
- W2017183118 cites W2135056130 @default.
- W2017183118 cites W2139377393 @default.
- W2017183118 cites W2142955857 @default.
- W2017183118 cites W2146941721 @default.
- W2017183118 cites W2148345396 @default.
- W2017183118 cites W2163494154 @default.
- W2017183118 cites W2164312004 @default.
- W2017183118 cites W2164395407 @default.
- W2017183118 cites W2167132834 @default.
- W2017183118 cites W2408815987 @default.
- W2017183118 cites W2418328677 @default.
- W2017183118 cites W315197452 @default.
- W2017183118 cites W4210579787 @default.
- W2017183118 doi "https://doi.org/10.1182/blood-2007-08-106153" @default.
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