FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4385012766> ?p ?o ?g. }

• W4385012766 endingPage "32" @default.
• W4385012766 startingPage "25" @default.
• W4385012766 abstract "Uncontrolled activation of the alternative pathway (AP) of complement, due to genetic and/or acquired defects, plays a primary pathogenetic role in C3 glomerulopathy (C3G), a rare and heterogeneous disease characterised by predominant C3 fragment deposition within the glomerulus, as well as glomerular damage. There are currently no approved disease-specific treatments for C3G, but new drugs that directly counteract AP dysregulation, targeting components of the pathway, have opened promising new perspectives for managing the disease. Complement factor B (FB), which is primarily synthesised by hepatocytes, is a key component of the AP, as it drives the central amplification loop of the complement system. In this study we used a GalNAc (N-Acetylgalactosamine)-conjugated siRNA to selectively target and suppress liver FB expression in two mouse models characterised by the complete (Cfh-/- mice) or partial (Cfh+/-) loss of function of complement factor H (FH). Homozygous deletion of FH induced a severe C3G phenotype, with strong dysregulation of the AP of complement, glomerular C3 deposition and almost complete C3 consumption. Mice with a heterozygous deletion of FH had intermediate C3 levels and exhibited slower disease progression, resembling human C3G more closely. Here we showed that FB siRNA treatment did not improve serum C3 levels, nor limit glomerular C3 deposition in Cfh-/- mice, while it did normalise circulating C3 levels, reduce glomerular C3 deposits, and limit mesangial electron-dense deposits in Cfh+/- mice. The present data provide important insights into the potential benefits and limitations of FB-targeted inhibition strategies and suggest RNA interference-mediated FB silencing in the liver as a possible therapeutic approach for treating C3G patients with FH haploinsufficiency." @default.
• W4385012766 created "2023-07-22" @default.
• W4385012766 creator A5001678867 @default.
• W4385012766 creator A5017028235 @default.
• W4385012766 creator A5019429551 @default.
• W4385012766 creator A5028151977 @default.
• W4385012766 creator A5035442609 @default.
• W4385012766 creator A5038695071 @default.
• W4385012766 creator A5041554168 @default.
• W4385012766 creator A5044705680 @default.
• W4385012766 creator A5054680461 @default.
• W4385012766 creator A5076316519 @default.
• W4385012766 creator A5088160070 @default.
• W4385012766 creator A5090115261 @default.
• W4385012766 date "2023-09-01" @default.
• W4385012766 modified "2023-10-12" @default.
• W4385012766 title "Liver factor B silencing to cure C3 glomerulopathy: Evidence from a mouse model of complement dysregulation" @default.
• W4385012766 cites W1576495961 @default.
• W4385012766 cites W1606415249 @default.
• W4385012766 cites W1980344266 @default.
• W4385012766 cites W1990372621 @default.
• W4385012766 cites W2041665769 @default.
• W4385012766 cites W2046033895 @default.
• W4385012766 cites W2056545631 @default.
• W4385012766 cites W2060433961 @default.
• W4385012766 cites W2065603844 @default.
• W4385012766 cites W2074286693 @default.
• W4385012766 cites W2077144266 @default.
• W4385012766 cites W2078890343 @default.
• W4385012766 cites W2084969272 @default.
• W4385012766 cites W2099871715 @default.
• W4385012766 cites W2116256426 @default.
• W4385012766 cites W2151241196 @default.
• W4385012766 cites W2775288206 @default.
• W4385012766 cites W2920438880 @default.
• W4385012766 cites W2927939151 @default.
• W4385012766 cites W2945507495 @default.
• W4385012766 cites W3022555156 @default.
• W4385012766 cites W3063613132 @default.
• W4385012766 cites W4289526307 @default.
• W4385012766 cites W4308834311 @default.
• W4385012766 doi "https://doi.org/10.1016/j.molimm.2023.07.010" @default.
• W4385012766 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/37481826" @default.
• W4385012766 hasPublicationYear "2023" @default.
• W4385012766 type Work @default.
• W4385012766 citedByCount "0" @default.
• W4385012766 crossrefType "journal-article" @default.
• W4385012766 hasAuthorship W4385012766A5001678867 @default.
• W4385012766 hasAuthorship W4385012766A5017028235 @default.
• W4385012766 hasAuthorship W4385012766A5019429551 @default.
• W4385012766 hasAuthorship W4385012766A5028151977 @default.
• W4385012766 hasAuthorship W4385012766A5035442609 @default.
• W4385012766 hasAuthorship W4385012766A5038695071 @default.
• W4385012766 hasAuthorship W4385012766A5041554168 @default.
• W4385012766 hasAuthorship W4385012766A5044705680 @default.
• W4385012766 hasAuthorship W4385012766A5054680461 @default.
• W4385012766 hasAuthorship W4385012766A5076316519 @default.
• W4385012766 hasAuthorship W4385012766A5088160070 @default.
• W4385012766 hasAuthorship W4385012766A5090115261 @default.
• W4385012766 hasBestOaLocation W43850127661 @default.
• W4385012766 hasConcept C104317684 @default.
• W4385012766 hasConcept C111684460 @default.
• W4385012766 hasConcept C119056186 @default.
• W4385012766 hasConcept C134018914 @default.
• W4385012766 hasConcept C14356644 @default.
• W4385012766 hasConcept C166703698 @default.
• W4385012766 hasConcept C179369057 @default.
• W4385012766 hasConcept C182629451 @default.
• W4385012766 hasConcept C189446657 @default.
• W4385012766 hasConcept C203014093 @default.
• W4385012766 hasConcept C22615655 @default.
• W4385012766 hasConcept C2777637130 @default.
• W4385012766 hasConcept C2779499704 @default.
• W4385012766 hasConcept C2780091579 @default.
• W4385012766 hasConcept C2780199430 @default.
• W4385012766 hasConcept C2780368995 @default.
• W4385012766 hasConcept C54009773 @default.
• W4385012766 hasConcept C54355233 @default.
• W4385012766 hasConcept C67705224 @default.
• W4385012766 hasConcept C86803240 @default.
• W4385012766 hasConcept C8891405 @default.
• W4385012766 hasConceptScore W4385012766C104317684 @default.
• W4385012766 hasConceptScore W4385012766C111684460 @default.
• W4385012766 hasConceptScore W4385012766C119056186 @default.
• W4385012766 hasConceptScore W4385012766C134018914 @default.
• W4385012766 hasConceptScore W4385012766C14356644 @default.
• W4385012766 hasConceptScore W4385012766C166703698 @default.
• W4385012766 hasConceptScore W4385012766C179369057 @default.
• W4385012766 hasConceptScore W4385012766C182629451 @default.
• W4385012766 hasConceptScore W4385012766C189446657 @default.
• W4385012766 hasConceptScore W4385012766C203014093 @default.
• W4385012766 hasConceptScore W4385012766C22615655 @default.
• W4385012766 hasConceptScore W4385012766C2777637130 @default.
• W4385012766 hasConceptScore W4385012766C2779499704 @default.
• W4385012766 hasConceptScore W4385012766C2780091579 @default.
• W4385012766 hasConceptScore W4385012766C2780199430 @default.
• W4385012766 hasConceptScore W4385012766C2780368995 @default.
• W4385012766 hasConceptScore W4385012766C54009773 @default.

• next