FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3083212105> ?p ?o ?g. }

• W3083212105 abstract "Abstract Background The type 1 interferon (IFN) response is part of the innate immune response and best known for its role in viral and bacterial infection. However, this pathway is also induced in sterile inflammation such as that which occurs in a number of neurodegenerative diseases, including neuronopathic Gaucher disease (nGD), a lysosomal storage disorder (LSD) caused by mutations in GBA . Methods Mice were injected with conduritol B-epoxide, an irreversible inhibitor of acid beta-glucosidase, the enzyme defective in nGD. MyTrMaSt null mice, where four adaptors of pathogen recognition receptors (PRRs) are deficient, were used to determine the role of the IFN pathway in nGD pathology. Activation of inflammatory and other pathways was analyzed by a variety of methods including RNAseq. Results Elevation in the expression of PRRs associated with the IFN response was observed in CBE-injected mice. Ablation of upstream pathways leading to IFN production had no therapeutic benefit on the lifespan of nGD mice but attenuated neuroinflammation. Primary and secondary pathological pathways (i.e., those associated or not with mouse survival) were distinguished, and a set of ~210 genes including those related to sphingolipid, cholesterol, and lipoprotein metabolism, along with a number of inflammatory pathways related to chemokines, TNF, TGF, complement, IL6, and damage-associated microglia were classified as primary pathological pathways, along with some lysosomal and neuronal genes. Conclusions Although IFN signaling is the top elevated pathway in nGD, we demonstrate that this pathway is not related to mouse viability and is consequently defined as a secondary pathology pathway. By elimination, we defined a number of critical pathways that are directly related to brain pathology in nGD, which in addition to its usefulness in understanding pathophysiological mechanisms, may also pave the way for the development of novel therapeutic paradigms by targeting such pathways." @default.
• W3083212105 created "2020-09-11" @default.
• W3083212105 creator A5008486712 @default.
• W3083212105 creator A5017198064 @default.
• W3083212105 creator A5051427079 @default.
• W3083212105 creator A5053401382 @default.
• W3083212105 creator A5057539969 @default.
• W3083212105 creator A5067203212 @default.
• W3083212105 date "2020-09-07" @default.
• W3083212105 modified "2023-10-16" @default.
• W3083212105 title "Mice defective in interferon signaling help distinguish between primary and secondary pathological pathways in a mouse model of neuronal forms of Gaucher disease" @default.
• W3083212105 cites W1576809668 @default.
• W3083212105 cites W1983323412 @default.
• W3083212105 cites W1995217653 @default.
• W3083212105 cites W2004366528 @default.
• W3083212105 cites W2009711029 @default.
• W3083212105 cites W2035258856 @default.
• W3083212105 cites W2071010503 @default.
• W3083212105 cites W2082399023 @default.
• W3083212105 cites W2091129202 @default.
• W3083212105 cites W2098898376 @default.
• W3083212105 cites W2102717949 @default.
• W3083212105 cites W2107277218 @default.
• W3083212105 cites W2115185894 @default.
• W3083212105 cites W2122914905 @default.
• W3083212105 cites W2133006561 @default.
• W3083212105 cites W2142512494 @default.
• W3083212105 cites W2163243965 @default.
• W3083212105 cites W2223511468 @default.
• W3083212105 cites W2297993206 @default.
• W3083212105 cites W2507117073 @default.
• W3083212105 cites W2559755333 @default.
• W3083212105 cites W2587657828 @default.
• W3083212105 cites W2754149889 @default.
• W3083212105 cites W2781955495 @default.
• W3083212105 cites W2789701709 @default.
• W3083212105 cites W2792364905 @default.
• W3083212105 cites W2806231203 @default.
• W3083212105 cites W2808503393 @default.
• W3083212105 cites W2902875229 @default.
• W3083212105 cites W2906703931 @default.
• W3083212105 cites W2920702628 @default.
• W3083212105 cites W2931036699 @default.
• W3083212105 cites W4241232520 @default.
• W3083212105 doi "https://doi.org/10.1186/s12974-020-01934-x" @default.
• W3083212105 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7487497" @default.
• W3083212105 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/32892753" @default.
• W3083212105 hasPublicationYear "2020" @default.
• W3083212105 type Work @default.
• W3083212105 sameAs 3083212105 @default.
• W3083212105 citedByCount "9" @default.
• W3083212105 countsByYear W30832121052021 @default.
• W3083212105 countsByYear W30832121052022 @default.
• W3083212105 countsByYear W30832121052023 @default.
• W3083212105 crossrefType "journal-article" @default.
• W3083212105 hasAuthorship W3083212105A5008486712 @default.
• W3083212105 hasAuthorship W3083212105A5017198064 @default.
• W3083212105 hasAuthorship W3083212105A5051427079 @default.
• W3083212105 hasAuthorship W3083212105A5053401382 @default.
• W3083212105 hasAuthorship W3083212105A5057539969 @default.
• W3083212105 hasAuthorship W3083212105A5067203212 @default.
• W3083212105 hasBestOaLocation W30832121051 @default.
• W3083212105 hasConcept C13373296 @default.
• W3083212105 hasConcept C136449434 @default.
• W3083212105 hasConcept C203014093 @default.
• W3083212105 hasConcept C2776178377 @default.
• W3083212105 hasConcept C2776914184 @default.
• W3083212105 hasConcept C2778690821 @default.
• W3083212105 hasConcept C2779830541 @default.
• W3083212105 hasConcept C61705674 @default.
• W3083212105 hasConcept C62478195 @default.
• W3083212105 hasConcept C86803240 @default.
• W3083212105 hasConcept C87753298 @default.
• W3083212105 hasConcept C8891405 @default.
• W3083212105 hasConcept C95444343 @default.
• W3083212105 hasConceptScore W3083212105C13373296 @default.
• W3083212105 hasConceptScore W3083212105C136449434 @default.
• W3083212105 hasConceptScore W3083212105C203014093 @default.
• W3083212105 hasConceptScore W3083212105C2776178377 @default.
• W3083212105 hasConceptScore W3083212105C2776914184 @default.
• W3083212105 hasConceptScore W3083212105C2778690821 @default.
• W3083212105 hasConceptScore W3083212105C2779830541 @default.
• W3083212105 hasConceptScore W3083212105C61705674 @default.
• W3083212105 hasConceptScore W3083212105C62478195 @default.
• W3083212105 hasConceptScore W3083212105C86803240 @default.
• W3083212105 hasConceptScore W3083212105C87753298 @default.
• W3083212105 hasConceptScore W3083212105C8891405 @default.
• W3083212105 hasConceptScore W3083212105C95444343 @default.
• W3083212105 hasIssue "1" @default.
• W3083212105 hasLocation W30832121051 @default.
• W3083212105 hasLocation W30832121052 @default.
• W3083212105 hasLocation W30832121053 @default.
• W3083212105 hasOpenAccess W3083212105 @default.
• W3083212105 hasPrimaryLocation W30832121051 @default.
• W3083212105 hasRelatedWork W2005666125 @default.
• W3083212105 hasRelatedWork W2040423913 @default.
• W3083212105 hasRelatedWork W2129708712 @default.
• W3083212105 hasRelatedWork W2622610015 @default.
• W3083212105 hasRelatedWork W2790494981 @default.
• W3083212105 hasRelatedWork W2889514518 @default.

• next