FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2988679683> ?p ?o ?g. }

• W2988679683 endingPage "343" @default.
• W2988679683 startingPage "323" @default.
• W2988679683 abstract "Secretory and cell membrane proteins are synthesized in the endoplasmic reticulum (ER), where a network of molecular chaperones and folding factors ensure correct protein folding and export to post‐ER compartments. Failure of this process leads to accumulation of unfolded/misfolded proteins, ER stress, and activation of the unfolded protein response (UPR), a complex signalling pathway aimed at restoring ER homeostasis, whose failure eventually leads to cell death. Suppressor of Ire1/Lhs1 double mutant (SIL1) is a nucleotide exchange factor for immunoglobulin binding protein, the main ER chaperone and primary sensor of ER stress. Loss of SIL1 function causes Marinesco‐Sjögren syndrome (MSS), a rare multisystem disease of early infancy for which there is no cure. This review, examines the current understanding of SIL1 activities in the ER, and reviews experimental data describing the consequences of SIL1 deficiency in cell and animal models. We discuss the evidence supporting a role of the UPR – particularly the protein kinase RNA‐like endoplasmic reticulum kinase branch – in the pathogenesis of MSS, and how this may be pharmacologically manipulated for treatment." @default.
• W2988679683 created "2019-11-22" @default.
• W2988679683 creator A5028263534 @default.
• W2988679683 creator A5030439171 @default.
• W2988679683 date "2020-05-18" @default.
• W2988679683 modified "2023-09-27" @default.
• W2988679683 title "Review: Protein misfolding diseases – the rare case of Marinesco‐Sjögren syndrome" @default.
• W2988679683 cites W1124350139 @default.
• W2988679683 cites W1884741867 @default.
• W2988679683 cites W1973528459 @default.
• W2988679683 cites W1975987422 @default.
• W2988679683 cites W1980618270 @default.
• W2988679683 cites W1985139784 @default.
• W2988679683 cites W1996367891 @default.
• W2988679683 cites W2000420976 @default.
• W2988679683 cites W2001756328 @default.
• W2988679683 cites W2003755766 @default.
• W2988679683 cites W2008845259 @default.
• W2988679683 cites W2010375635 @default.
• W2988679683 cites W2023971182 @default.
• W2988679683 cites W2026376387 @default.
• W2988679683 cites W2029499699 @default.
• W2988679683 cites W2030221208 @default.
• W2988679683 cites W2036604732 @default.
• W2988679683 cites W2044241702 @default.
• W2988679683 cites W2045534807 @default.
• W2988679683 cites W2051467270 @default.
• W2988679683 cites W2064436957 @default.
• W2988679683 cites W2065355192 @default.
• W2988679683 cites W2066461267 @default.
• W2988679683 cites W2067962322 @default.
• W2988679683 cites W2070591805 @default.
• W2988679683 cites W2079088912 @default.
• W2988679683 cites W2086723377 @default.
• W2988679683 cites W2086795878 @default.
• W2988679683 cites W2087932478 @default.
• W2988679683 cites W2089198803 @default.
• W2988679683 cites W2089337481 @default.
• W2988679683 cites W2096915343 @default.
• W2988679683 cites W2100669458 @default.
• W2988679683 cites W2104772632 @default.
• W2988679683 cites W2112682006 @default.
• W2988679683 cites W2115988944 @default.
• W2988679683 cites W2117581921 @default.
• W2988679683 cites W2119506154 @default.
• W2988679683 cites W2123785861 @default.
• W2988679683 cites W2128021875 @default.
• W2988679683 cites W2131450597 @default.
• W2988679683 cites W2136359337 @default.
• W2988679683 cites W2140731000 @default.
• W2988679683 cites W2151221425 @default.
• W2988679683 cites W2186821278 @default.
• W2988679683 cites W2386603878 @default.
• W2988679683 cites W2540842333 @default.
• W2988679683 cites W2593892721 @default.
• W2988679683 cites W2605803966 @default.
• W2988679683 cites W2606749138 @default.
• W2988679683 cites W2687861173 @default.
• W2988679683 cites W2740706732 @default.
• W2988679683 cites W2773112660 @default.
• W2988679683 cites W2793107637 @default.
• W2988679683 cites W2798145931 @default.
• W2988679683 cites W2800343508 @default.
• W2988679683 cites W2831186540 @default.
• W2988679683 cites W2900569176 @default.
• W2988679683 cites W2901474922 @default.
• W2988679683 cites W2901527668 @default.
• W2988679683 cites W4232821763 @default.
• W2988679683 doi "https://doi.org/10.1111/nan.12588" @default.
• W2988679683 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31701543" @default.
• W2988679683 hasPublicationYear "2020" @default.
• W2988679683 type Work @default.
• W2988679683 sameAs 2988679683 @default.
• W2988679683 citedByCount "3" @default.
• W2988679683 countsByYear W29886796832020 @default.
• W2988679683 countsByYear W29886796832021 @default.
• W2988679683 crossrefType "journal-article" @default.
• W2988679683 hasAuthorship W2988679683A5028263534 @default.
• W2988679683 hasAuthorship W2988679683A5030439171 @default.
• W2988679683 hasConcept C139447449 @default.
• W2988679683 hasConcept C142724271 @default.
• W2988679683 hasConcept C158617107 @default.
• W2988679683 hasConcept C158619295 @default.
• W2988679683 hasConcept C184235292 @default.
• W2988679683 hasConcept C185592680 @default.
• W2988679683 hasConcept C204328495 @default.
• W2988679683 hasConcept C2775962898 @default.
• W2988679683 hasConcept C71924100 @default.
• W2988679683 hasConcept C86803240 @default.
• W2988679683 hasConcept C95444343 @default.
• W2988679683 hasConcept C97029542 @default.
• W2988679683 hasConceptScore W2988679683C139447449 @default.
• W2988679683 hasConceptScore W2988679683C142724271 @default.
• W2988679683 hasConceptScore W2988679683C158617107 @default.
• W2988679683 hasConceptScore W2988679683C158619295 @default.
• W2988679683 hasConceptScore W2988679683C184235292 @default.
• W2988679683 hasConceptScore W2988679683C185592680 @default.
• W2988679683 hasConceptScore W2988679683C204328495 @default.

• next