Matches in SemOpenAlex for { <https://semopenalex.org/work/W2000997929> ?p ?o ?g. }
- W2000997929 endingPage "120" @default.
- W2000997929 startingPage "115" @default.
- W2000997929 abstract "Mutations in CLN3 gene cause juvenile neuronal ceroid lipofuscinosis (JNCL or Batten disease), an early-onset neurodegenerative disorder that is characterized by the accumulation of ceroid lipofuscin within lysosomes. The function of the CLN3 protein remains unclear and is presumed to be related to Endoplasmic reticulum (ER) stress. To investigate the function of CLN3 in the ER stress signaling pathway, we measured proliferation and apoptosis in cells transfected with normal and mutant CLN3 after treatment with the ER stress inducer tunicamycin (TM). We found that overexpression of CLN3 was sufficient in conferring increased resistance to ER stress. Wild-type CLN3 protected cells from TM-induced apoptosis and increased cell proliferation. Overexpression of wild-type CLN3 enhanced expression of the ER chaperone protein, glucose-regulated protein 78 (GRP78), and reduced expression of the proapoptotic protein CCAAT/-enhancer-binding protein homologous protein (CHOP). In contrast, overexpression of mutant CLN3 or siRNA knockdown of CLN3 produced the opposite effect. Together, our data suggest that the lack of CLN3 function in cells leads to a failure of management in the response to ER stress and this may be the key deficit in JNCL that causes neuronal degeneration." @default.
- W2000997929 created "2016-06-24" @default.
- W2000997929 creator A5009106787 @default.
- W2000997929 creator A5035282947 @default.
- W2000997929 creator A5036476031 @default.
- W2000997929 creator A5047376512 @default.
- W2000997929 creator A5052600624 @default.
- W2000997929 creator A5062451164 @default.
- W2000997929 date "2014-04-01" @default.
- W2000997929 modified "2023-10-17" @default.
- W2000997929 title "The Batten disease gene CLN3 confers resistance to endoplasmic reticulum stress induced by tunicamycin" @default.
- W2000997929 cites W1550967330 @default.
- W2000997929 cites W1971108880 @default.
- W2000997929 cites W1977206684 @default.
- W2000997929 cites W1980162202 @default.
- W2000997929 cites W2001491246 @default.
- W2000997929 cites W2051479964 @default.
- W2000997929 cites W2061867766 @default.
- W2000997929 cites W2062619795 @default.
- W2000997929 cites W2063918436 @default.
- W2000997929 cites W2065926476 @default.
- W2000997929 cites W2078039611 @default.
- W2000997929 cites W2087516011 @default.
- W2000997929 cites W2088234686 @default.
- W2000997929 cites W2096966529 @default.
- W2000997929 cites W2112625478 @default.
- W2000997929 cites W2114022615 @default.
- W2000997929 cites W2115679330 @default.
- W2000997929 cites W2122666486 @default.
- W2000997929 cites W2132596442 @default.
- W2000997929 cites W2138812188 @default.
- W2000997929 cites W2144827966 @default.
- W2000997929 cites W2146870223 @default.
- W2000997929 cites W2149518951 @default.
- W2000997929 cites W2160879813 @default.
- W2000997929 cites W2161217327 @default.
- W2000997929 cites W4246806002 @default.
- W2000997929 cites W4361773848 @default.
- W2000997929 doi "https://doi.org/10.1016/j.bbrc.2014.03.120" @default.
- W2000997929 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/24699413" @default.
- W2000997929 hasPublicationYear "2014" @default.
- W2000997929 type Work @default.
- W2000997929 sameAs 2000997929 @default.
- W2000997929 citedByCount "12" @default.
- W2000997929 countsByYear W20009979292015 @default.
- W2000997929 countsByYear W20009979292017 @default.
- W2000997929 countsByYear W20009979292018 @default.
- W2000997929 countsByYear W20009979292019 @default.
- W2000997929 countsByYear W20009979292020 @default.
- W2000997929 countsByYear W20009979292022 @default.
- W2000997929 countsByYear W20009979292023 @default.
- W2000997929 crossrefType "journal-article" @default.
- W2000997929 hasAuthorship W2000997929A5009106787 @default.
- W2000997929 hasAuthorship W2000997929A5035282947 @default.
- W2000997929 hasAuthorship W2000997929A5036476031 @default.
- W2000997929 hasAuthorship W2000997929A5047376512 @default.
- W2000997929 hasAuthorship W2000997929A5052600624 @default.
- W2000997929 hasAuthorship W2000997929A5062451164 @default.
- W2000997929 hasConcept C104317684 @default.
- W2000997929 hasConcept C139447449 @default.
- W2000997929 hasConcept C153911025 @default.
- W2000997929 hasConcept C158617107 @default.
- W2000997929 hasConcept C2777575526 @default.
- W2000997929 hasConcept C2778230176 @default.
- W2000997929 hasConcept C54009773 @default.
- W2000997929 hasConcept C55493867 @default.
- W2000997929 hasConcept C86803240 @default.
- W2000997929 hasConcept C95444343 @default.
- W2000997929 hasConceptScore W2000997929C104317684 @default.
- W2000997929 hasConceptScore W2000997929C139447449 @default.
- W2000997929 hasConceptScore W2000997929C153911025 @default.
- W2000997929 hasConceptScore W2000997929C158617107 @default.
- W2000997929 hasConceptScore W2000997929C2777575526 @default.
- W2000997929 hasConceptScore W2000997929C2778230176 @default.
- W2000997929 hasConceptScore W2000997929C54009773 @default.
- W2000997929 hasConceptScore W2000997929C55493867 @default.
- W2000997929 hasConceptScore W2000997929C86803240 @default.
- W2000997929 hasConceptScore W2000997929C95444343 @default.
- W2000997929 hasFunder F4320321001 @default.
- W2000997929 hasIssue "1" @default.
- W2000997929 hasLocation W20009979291 @default.
- W2000997929 hasLocation W20009979292 @default.
- W2000997929 hasOpenAccess W2000997929 @default.
- W2000997929 hasPrimaryLocation W20009979291 @default.
- W2000997929 hasRelatedWork W2013521084 @default.
- W2000997929 hasRelatedWork W2031088196 @default.
- W2000997929 hasRelatedWork W2060719045 @default.
- W2000997929 hasRelatedWork W2064194904 @default.
- W2000997929 hasRelatedWork W2086116768 @default.
- W2000997929 hasRelatedWork W2129786365 @default.
- W2000997929 hasRelatedWork W2374240884 @default.
- W2000997929 hasRelatedWork W2416524069 @default.
- W2000997929 hasRelatedWork W2754689301 @default.
- W2000997929 hasRelatedWork W3030241489 @default.
- W2000997929 hasVolume "447" @default.
- W2000997929 isParatext "false" @default.
- W2000997929 isRetracted "false" @default.
- W2000997929 magId "2000997929" @default.