FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3202621396> ?p ?o ?g. }

• W3202621396 endingPage "10752" @default.
• W3202621396 startingPage "10752" @default.
• W3202621396 abstract "Aggregation of β2 microglobulin (β2m) into amyloid fibrils is associated with systemic amyloidosis, caused by the deposition of amyloid fibrils containing the wild-type protein and its truncated variant, ΔN6 β2m, in haemo-dialysed patients. A second form of familial systemic amyloidosis caused by the β2m variant, D76N, results in amyloid deposits in the viscera, without renal dysfunction. Although the folding and misfolding mechanisms of β2 microglobulin have been widely studied in vitro and in vivo, we lack a comparable understanding of the molecular mechanisms underlying toxicity in a cellular and organismal environment. Here, we established transgenic C. elegans lines expressing wild-type (WT) human β2m, or the two highly amyloidogenic naturally occurring variants, D76N β2m and ΔN6 β2m, in the C. elegans bodywall muscle. Nematodes expressing the D76N β2m and ΔN6 β2m variants exhibit increased age-dependent and cell nonautonomous proteotoxicity associated with reduced motility, delayed development and shortened lifespan. Both β2m variants cause widespread endogenous protein aggregation contributing to the increased toxicity in aged animals. We show that expression of β2m reduces the capacity of C. elegans to cope with heat and endoplasmic reticulum (ER) stress, correlating with a deficiency to upregulate BiP/hsp-4 transcripts in response to ER stress in young adult animals. Interestingly, protein secretion in all β2m variants is reduced, despite the presence of the natural signal sequence, suggesting a possible link between organismal β2m toxicity and a disrupted ER secretory metabolism." @default.
• W3202621396 created "2021-10-11" @default.
• W3202621396 creator A5019410249 @default.
• W3202621396 creator A5066535665 @default.
• W3202621396 creator A5074934123 @default.
• W3202621396 creator A5087275613 @default.
• W3202621396 date "2021-10-04" @default.
• W3202621396 modified "2023-09-27" @default.
• W3202621396 title "Global Proteotoxicity Caused by Human β2 Microglobulin Variants Impairs the Unfolded Protein Response in C. elegans" @default.
• W3202621396 cites W1697178647 @default.
• W3202621396 cites W1823713625 @default.
• W3202621396 cites W1944127002 @default.
• W3202621396 cites W1965754110 @default.
• W3202621396 cites W1970225577 @default.
• W3202621396 cites W1970500983 @default.
• W3202621396 cites W1970577444 @default.
• W3202621396 cites W1977603207 @default.
• W3202621396 cites W1984589081 @default.
• W3202621396 cites W1986060177 @default.
• W3202621396 cites W1986737010 @default.
• W3202621396 cites W1989383469 @default.
• W3202621396 cites W1990695557 @default.
• W3202621396 cites W2001155578 @default.
• W3202621396 cites W2002439304 @default.
• W3202621396 cites W2004085006 @default.
• W3202621396 cites W2012387790 @default.
• W3202621396 cites W2013429682 @default.
• W3202621396 cites W2013513784 @default.
• W3202621396 cites W2026306293 @default.
• W3202621396 cites W2030843640 @default.
• W3202621396 cites W2031998484 @default.
• W3202621396 cites W2034057547 @default.
• W3202621396 cites W2037911961 @default.
• W3202621396 cites W2040359453 @default.
• W3202621396 cites W2051359509 @default.
• W3202621396 cites W2053885309 @default.
• W3202621396 cites W2055439148 @default.
• W3202621396 cites W2070751693 @default.
• W3202621396 cites W2076159058 @default.
• W3202621396 cites W2099496568 @default.
• W3202621396 cites W2115971557 @default.
• W3202621396 cites W2119596051 @default.
• W3202621396 cites W2144617447 @default.
• W3202621396 cites W2154545577 @default.
• W3202621396 cites W2156707027 @default.
• W3202621396 cites W2166696251 @default.
• W3202621396 cites W2167688505 @default.
• W3202621396 cites W2192080449 @default.
• W3202621396 cites W2201593523 @default.
• W3202621396 cites W2297273877 @default.
• W3202621396 cites W2344074248 @default.
• W3202621396 cites W2515386838 @default.
• W3202621396 cites W2531576435 @default.
• W3202621396 cites W2750210509 @default.
• W3202621396 cites W2810557289 @default.
• W3202621396 cites W2890980244 @default.
• W3202621396 cites W2913940235 @default.
• W3202621396 cites W2940040548 @default.
• W3202621396 cites W2951221898 @default.
• W3202621396 cites W2996860114 @default.
• W3202621396 cites W2998280309 @default.
• W3202621396 cites W3041873466 @default.
• W3202621396 cites W4230140780 @default.
• W3202621396 doi "https://doi.org/10.3390/ijms221910752" @default.
• W3202621396 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/8509642" @default.
• W3202621396 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/34639093" @default.
• W3202621396 hasPublicationYear "2021" @default.
• W3202621396 type Work @default.
• W3202621396 sameAs 3202621396 @default.
• W3202621396 citedByCount "3" @default.
• W3202621396 countsByYear W32026213962022 @default.
• W3202621396 countsByYear W32026213962023 @default.
• W3202621396 crossrefType "journal-article" @default.
• W3202621396 hasAuthorship W3202621396A5019410249 @default.
• W3202621396 hasAuthorship W3202621396A5066535665 @default.
• W3202621396 hasAuthorship W3202621396A5074934123 @default.
• W3202621396 hasAuthorship W3202621396A5087275613 @default.
• W3202621396 hasBestOaLocation W32026213961 @default.
• W3202621396 hasConcept C102230213 @default.
• W3202621396 hasConcept C104317684 @default.
• W3202621396 hasConcept C126322002 @default.
• W3202621396 hasConcept C136238340 @default.
• W3202621396 hasConcept C139447449 @default.
• W3202621396 hasConcept C141035611 @default.
• W3202621396 hasConcept C143065580 @default.
• W3202621396 hasConcept C158617107 @default.
• W3202621396 hasConcept C178790620 @default.
• W3202621396 hasConcept C185592680 @default.
• W3202621396 hasConcept C203014093 @default.
• W3202621396 hasConcept C204328495 @default.
• W3202621396 hasConcept C207583985 @default.
• W3202621396 hasConcept C21957315 @default.
• W3202621396 hasConcept C2776104650 @default.
• W3202621396 hasConcept C2777633098 @default.
• W3202621396 hasConcept C2778944004 @default.
• W3202621396 hasConcept C2779951007 @default.
• W3202621396 hasConcept C29730261 @default.
• W3202621396 hasConcept C55493867 @default.

• next