Matches in SemOpenAlex for { <https://semopenalex.org/work/W2091223832> ?p ?o ?g. }
- W2091223832 endingPage "3337" @default.
- W2091223832 startingPage "3331" @default.
- W2091223832 abstract "Leber hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by visual loss resulting from retinal ganglion cell degeneration. Despite the important role of respiratory chain deficiency and oxidative stress induced by mtDNA point mutations affecting complex I, excitotoxic injury has been postulated as a concurrent pathogenic factor. We used transmitochondrial cybrid cell lines constructed using enucleated fibroblasts from three LHON probands carrying the most severe 3460/ND1 mutation and three controls as mitochondria donors, and the osteosarcoma-derived mtDNA-less cells, to study the possible efficacy of two selected antioxidant compounds in preventing glutamate uptake reduction previously observed in LHON cybrids. We demonstrated that two antioxidants, Trolox and decylubiquinone, partially restore glutamate transport impairment occurring in LHON cybrids. Rotenone, a classic complex I inhibitor, did not worsen the glutamate uptake defect present in LHON cybrids under basal conditions but significantly reduced glutamate transport in control cybrids. Furthermore, we observed that LHON cybrids showed an increased protein carbonylation under basal conditions, not further affected by rotenone and partially counteracted by antioxidants. Our findings strengthen the hypothesis that the complex I defect associated with LHON causes free radical overproduction, which is responsible for glutamate transport inhibition. We suggest that selected antioxidants may be clinically tested in LHON patients and relatives to restore glutamate uptake defect caused by LHON-associated free radical overproduction. © 2008 Wiley-Liss, Inc." @default.
- W2091223832 created "2016-06-24" @default.
- W2091223832 creator A5010090990 @default.
- W2091223832 creator A5016027674 @default.
- W2091223832 creator A5025574771 @default.
- W2091223832 creator A5035089440 @default.
- W2091223832 creator A5036154881 @default.
- W2091223832 creator A5043413367 @default.
- W2091223832 creator A5064357836 @default.
- W2091223832 creator A5065012064 @default.
- W2091223832 creator A5070622522 @default.
- W2091223832 creator A5086297781 @default.
- W2091223832 date "2008-11-15" @default.
- W2091223832 modified "2023-10-07" @default.
- W2091223832 title "Antioxidants partially restore glutamate transport defect in leber hereditary optic neuropathy cybrids" @default.
- W2091223832 cites W1516411633 @default.
- W2091223832 cites W1594802398 @default.
- W2091223832 cites W1894069867 @default.
- W2091223832 cites W1963894732 @default.
- W2091223832 cites W1974779272 @default.
- W2091223832 cites W1977510154 @default.
- W2091223832 cites W2001083394 @default.
- W2091223832 cites W2015808639 @default.
- W2091223832 cites W2016163301 @default.
- W2091223832 cites W2017692041 @default.
- W2091223832 cites W2018302460 @default.
- W2091223832 cites W2025032554 @default.
- W2091223832 cites W2026944055 @default.
- W2091223832 cites W2048965827 @default.
- W2091223832 cites W2050270081 @default.
- W2091223832 cites W2053710386 @default.
- W2091223832 cites W2055595633 @default.
- W2091223832 cites W2057008816 @default.
- W2091223832 cites W2058837644 @default.
- W2091223832 cites W2067124266 @default.
- W2091223832 cites W2071888455 @default.
- W2091223832 cites W2095445944 @default.
- W2091223832 cites W2107396020 @default.
- W2091223832 cites W2109645784 @default.
- W2091223832 cites W2119201576 @default.
- W2091223832 cites W2141981416 @default.
- W2091223832 cites W2164271893 @default.
- W2091223832 cites W2167480986 @default.
- W2091223832 cites W2169355774 @default.
- W2091223832 doi "https://doi.org/10.1002/jnr.21773" @default.
- W2091223832 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/18615737" @default.
- W2091223832 hasPublicationYear "2008" @default.
- W2091223832 type Work @default.
- W2091223832 sameAs 2091223832 @default.
- W2091223832 citedByCount "22" @default.
- W2091223832 countsByYear W20912238322012 @default.
- W2091223832 countsByYear W20912238322014 @default.
- W2091223832 countsByYear W20912238322015 @default.
- W2091223832 countsByYear W20912238322016 @default.
- W2091223832 countsByYear W20912238322017 @default.
- W2091223832 countsByYear W20912238322019 @default.
- W2091223832 countsByYear W20912238322020 @default.
- W2091223832 countsByYear W20912238322021 @default.
- W2091223832 countsByYear W20912238322023 @default.
- W2091223832 crossrefType "journal-article" @default.
- W2091223832 hasAuthorship W2091223832A5010090990 @default.
- W2091223832 hasAuthorship W2091223832A5016027674 @default.
- W2091223832 hasAuthorship W2091223832A5025574771 @default.
- W2091223832 hasAuthorship W2091223832A5035089440 @default.
- W2091223832 hasAuthorship W2091223832A5036154881 @default.
- W2091223832 hasAuthorship W2091223832A5043413367 @default.
- W2091223832 hasAuthorship W2091223832A5064357836 @default.
- W2091223832 hasAuthorship W2091223832A5065012064 @default.
- W2091223832 hasAuthorship W2091223832A5070622522 @default.
- W2091223832 hasAuthorship W2091223832A5086297781 @default.
- W2091223832 hasConcept C104317684 @default.
- W2091223832 hasConcept C105702510 @default.
- W2091223832 hasConcept C170493617 @default.
- W2091223832 hasConcept C176944494 @default.
- W2091223832 hasConcept C185592680 @default.
- W2091223832 hasConcept C2776151105 @default.
- W2091223832 hasConcept C2776231299 @default.
- W2091223832 hasConcept C2777056713 @default.
- W2091223832 hasConcept C2778233873 @default.
- W2091223832 hasConcept C2778389096 @default.
- W2091223832 hasConcept C2780837183 @default.
- W2091223832 hasConcept C28859421 @default.
- W2091223832 hasConcept C2993539680 @default.
- W2091223832 hasConcept C3020377403 @default.
- W2091223832 hasConcept C501734568 @default.
- W2091223832 hasConcept C54355233 @default.
- W2091223832 hasConcept C55493867 @default.
- W2091223832 hasConcept C61174792 @default.
- W2091223832 hasConcept C86803240 @default.
- W2091223832 hasConceptScore W2091223832C104317684 @default.
- W2091223832 hasConceptScore W2091223832C105702510 @default.
- W2091223832 hasConceptScore W2091223832C170493617 @default.
- W2091223832 hasConceptScore W2091223832C176944494 @default.
- W2091223832 hasConceptScore W2091223832C185592680 @default.
- W2091223832 hasConceptScore W2091223832C2776151105 @default.
- W2091223832 hasConceptScore W2091223832C2776231299 @default.
- W2091223832 hasConceptScore W2091223832C2777056713 @default.
- W2091223832 hasConceptScore W2091223832C2778233873 @default.