| http://purl.uniprot.org/citations/29858280 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/29858280 | http://www.w3.org/2000/01/rdf-schema#comment | "Background Properdin (P) is a positive regulator of the alternative pathway of complement activation. Although P inhibition is expected and has been shown to ameliorate the alternative pathway of complement-mediated tissue injury in several disease models, it unexpectedly exacerbated renal injury in a murine model of C3 glomerulopathy. The role of P in atypical hemolytic uremic syndrome (aHUS) is uncertain.Methods We blocked P function by genetic deletion or mAb-mediated inhibition in mice carrying a factor H (FH) point mutation, W1206R (FHR/R), that causes aHUS and systemic thrombophilia with high mortality.Results P deficiency completely rescued FHR/R mice from premature death and prevented thrombocytopenia, hemolytic anemia, and renal disease. It also eliminated macrovessel thrombi that were prevalent in FHR/R mice. All mice that received a function-blocking anti-P mAb for 8 weeks survived the experimental period and appeared grossly healthy. Platelet counts and hemoglobin levels were significantly improved in FHR/R mice after 4 weeks of anti-P mAb treatment. One half of the FHR/R mice treated with an isotype control mAb but none of the anti-P mAb-treated mice developed stroke-related neurologic disease. Anti-P mAb-treated FHR/R mice showed largely normal renal histology, and residual liver thrombi were detected in only three of 15 treated mice.Conclusions These results contrast with the detrimental effect of P inhibition observed in a murine model of C3 glomerulopathy and suggest that P contributes critically to aHUS pathogenesis. Inhibition of P in aHUS may be of therapeutic benefit."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.org/dc/terms/identifier | "doi:10.1681/asn.2017121244"xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Kim H."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Palmer M."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Sato S."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Ueda Y."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Miwa T."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Ito D."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Song W.C."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/author | "Gullipalli D."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/date | "2018"xsd:gYear |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/name | "J Am Soc Nephrol"xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/pages | "1928-1937"xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/title | "Blocking Properdin Prevents Complement-Mediated Hemolytic Uremic Syndrome and Systemic Thrombophilia."xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://purl.uniprot.org/core/volume | "29"xsd:string |
| http://purl.uniprot.org/citations/29858280 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/29858280 |
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