期刊
JOURNAL OF IMMUNOLOGY
卷 169, 期 9, 页码 4702-4706出版社
AMER ASSOC IMMUNOLOGISTS
DOI: 10.4049/jimmunol.169.9.4702
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资金
- NIDDK NIH HHS [DK-35081] Funding Source: Medline
Incidents of hemolytic uremic syndrome (HUS) include a subset of patients that exhibit mutations in C factor H. These mutations cluster in the C-terminal domains of factor H where previous reports have identified polyanion and CA-binding sites. In this study, we show that recombinant human factor H with deletions at the C-terminal end of the protein loses the ability to control the spontaneous activation of the alternative C pathway on host-like surfaces. For the pathology of HUS, the findings imply that mutations that disrupt the normal functions of the C-terminal domains prevent host polyanion recognition. The resulting uncontrolled activation of complement on susceptible host tissues appears to be the initiating event behind the acute renal failure of familial HUS patients.
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