FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2991366148> ?p ?o ?g. }

• W2991366148 endingPage "118613" @default.
• W2991366148 startingPage "118613" @default.
• W2991366148 abstract "Lafora progressive myoclonus epilepsy is a fatal rare neurodegenerative disorder characterized by the accumulation of insoluble abnormal glycogen deposits in the brain and peripheral tissues. Mutations in at least two genes are responsible for the disease: EPM2A , encoding the glucan phosphatase laforin, and EPM2B , encoding the RING-type E3-ubiquitin ligase malin. Both laforin and malin form a functional complex in which laforin recruits the substrates to be ubiquitinated by malin. We and others have described that, in cellular and animal models of this disease, there is an autophagy impairment which leads to the accumulation of dysfunctional mitochondria. In addition, we established that the autophagic defect occurred at the initial steps of autophagosome formation. In this work, we present evidence that in cellular models of the disease there is a decrease in the amount of phosphatidylinositol-3P. This is probably due to defective regulation of the autophagic PI3KC3 complex, in the absence of a functional laforin/malin complex. In fact, we demonstrate that the laforin/malin complex interacts physically and co-localizes intracellularly with core components of the PI3KC3 complex (Beclin1, Vps34 and Vps15), and that this interaction is specific and results in the polyubiquitination of these proteins. In addition, the laforin/malin complex is also able to polyubiquitinate ATG14L and UVRAG. Finally, we show that overexpression of the laforin/malin complex increases PI3KC3 activity. All these results suggest a new role of the laforin/malin complex in the activation of autophagy via regulation of the PI3KC3 complex and explain the defect in autophagy described in Lafora disease. • Lafora disease cell models produce lower levels of PI3P. • The laforin/malin E3 ligase interacts and ubiquitinates Beclin1. • The laforin/malin complex interacts with additional components of the PI3KC3 complex. • Ubiquitination of the PI3KC3 complex by laforin and malin increases its activity." @default.
• W2991366148 created "2019-12-05" @default.
• W2991366148 creator A5040017248 @default.
• W2991366148 creator A5040225569 @default.
• W2991366148 creator A5052293068 @default.
• W2991366148 creator A5062041886 @default.
• W2991366148 creator A5086960324 @default.
• W2991366148 date "2020-02-01" @default.
• W2991366148 modified "2023-09-23" @default.
• W2991366148 title "Regulation of the autophagic PI3KC3 complex by laforin/malin E3-ubiquitin ligase, two proteins involved in Lafora disease" @default.
• W2991366148 cites W1499183463 @default.
• W2991366148 cites W1584056801 @default.
• W2991366148 cites W1900548871 @default.
• W2991366148 cites W1979339081 @default.
• W2991366148 cites W1979529568 @default.
• W2991366148 cites W1982962675 @default.
• W2991366148 cites W1983220029 @default.
• W2991366148 cites W1985790212 @default.
• W2991366148 cites W1997353282 @default.
• W2991366148 cites W2016631802 @default.
• W2991366148 cites W2067490363 @default.
• W2991366148 cites W2071519991 @default.
• W2991366148 cites W2080352858 @default.
• W2991366148 cites W2110601384 @default.
• W2991366148 cites W2115632183 @default.
• W2991366148 cites W2126722763 @default.
• W2991366148 cites W2131712753 @default.
• W2991366148 cites W2131872983 @default.
• W2991366148 cites W2137181909 @default.
• W2991366148 cites W2140641604 @default.
• W2991366148 cites W2145780431 @default.
• W2991366148 cites W2153524819 @default.
• W2991366148 cites W2164924935 @default.
• W2991366148 cites W2167881747 @default.
• W2991366148 cites W2174370490 @default.
• W2991366148 cites W2340549461 @default.
• W2991366148 cites W2528440934 @default.
• W2991366148 cites W2571114420 @default.
• W2991366148 cites W2592067246 @default.
• W2991366148 cites W2604240481 @default.
• W2991366148 cites W2621728938 @default.
• W2991366148 cites W2745425348 @default.
• W2991366148 cites W2763871199 @default.
• W2991366148 cites W2766944946 @default.
• W2991366148 cites W2768840828 @default.
• W2991366148 cites W2770522282 @default.
• W2991366148 cites W2794915225 @default.
• W2991366148 cites W2795845448 @default.
• W2991366148 cites W2797279856 @default.
• W2991366148 cites W2883006983 @default.
• W2991366148 cites W2905006309 @default.
• W2991366148 cites W4253524409 @default.
• W2991366148 doi "https://doi.org/10.1016/j.bbamcr.2019.118613" @default.
• W2991366148 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6937399" @default.
• W2991366148 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31758957" @default.
• W2991366148 hasPublicationYear "2020" @default.
• W2991366148 type Work @default.
• W2991366148 sameAs 2991366148 @default.
• W2991366148 citedByCount "18" @default.
• W2991366148 countsByYear W29913661482020 @default.
• W2991366148 countsByYear W29913661482021 @default.
• W2991366148 countsByYear W29913661482022 @default.
• W2991366148 countsByYear W29913661482023 @default.
• W2991366148 crossrefType "journal-article" @default.
• W2991366148 hasAuthorship W2991366148A5040017248 @default.
• W2991366148 hasAuthorship W2991366148A5040225569 @default.
• W2991366148 hasAuthorship W2991366148A5052293068 @default.
• W2991366148 hasAuthorship W2991366148A5062041886 @default.
• W2991366148 hasAuthorship W2991366148A5086960324 @default.
• W2991366148 hasBestOaLocation W29913661481 @default.
• W2991366148 hasConcept C104317684 @default.
• W2991366148 hasConcept C11960822 @default.
• W2991366148 hasConcept C134459356 @default.
• W2991366148 hasConcept C178666793 @default.
• W2991366148 hasConcept C185592680 @default.
• W2991366148 hasConcept C190283241 @default.
• W2991366148 hasConcept C203522944 @default.
• W2991366148 hasConcept C25602115 @default.
• W2991366148 hasConcept C2777280662 @default.
• W2991366148 hasConcept C55493867 @default.
• W2991366148 hasConcept C86803240 @default.
• W2991366148 hasConcept C95444343 @default.
• W2991366148 hasConceptScore W2991366148C104317684 @default.
• W2991366148 hasConceptScore W2991366148C11960822 @default.
• W2991366148 hasConceptScore W2991366148C134459356 @default.
• W2991366148 hasConceptScore W2991366148C178666793 @default.
• W2991366148 hasConceptScore W2991366148C185592680 @default.
• W2991366148 hasConceptScore W2991366148C190283241 @default.
• W2991366148 hasConceptScore W2991366148C203522944 @default.
• W2991366148 hasConceptScore W2991366148C25602115 @default.
• W2991366148 hasConceptScore W2991366148C2777280662 @default.
• W2991366148 hasConceptScore W2991366148C55493867 @default.
• W2991366148 hasConceptScore W2991366148C86803240 @default.
• W2991366148 hasConceptScore W2991366148C95444343 @default.
• W2991366148 hasFunder F4320332161 @default.
• W2991366148 hasIssue "2" @default.
• W2991366148 hasLocation W29913661481 @default.
• W2991366148 hasLocation W29913661482 @default.

• next