FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2064707684> ?p ?o ?g. }

• W2064707684 endingPage "653" @default.
• W2064707684 startingPage "645" @default.
• W2064707684 abstract "Wilson's disease results from mutations in the P-type Cu(2+)-ATPase causing Cu(2+) toxicity. We previously demonstrated that exposure of mixed neuronal/glial cultures to 20 microM Cu(2+) induced ATP loss and death that were attenuated by mitochondrial substrates, activators, and cofactors. Here, we show differential cellular sensitivity to Cu(2+) that was equalized to 5 microM in the presence of the copper exchanger/ionophore, disulfiram. Because Cu(2+) facilitates formation of oxygen radicals (ROS) which inhibit pyruvate dehydrogenase (PDH) and alpha-ketoglutarate dehydrogenase (KGDH), we hypothesized that their inhibition contributed to Cu(2+)-induced death. Toxic CU(2+) exposure was accompanied by early inhibition of neuronal and hepatocellular PDH and KGDH activities, followed by reduced mitochondrial transmembrane potential, DeltaPsi(M). Thiamine (1-6 mM), and dihydrolipoic acid (LA, 50 microM), required cofactors for PDH and KGDH, attenuated this enzymatic inhibition and subsequent death in all cell types. Furthermore, liver PDH and KGDH activities were reduced in the Atp7b mouse model of Wilson's disease prior to liver damage, and were partially restored by oral thiamine supplementation. These data support our hypothesis that Cu(2+)-induced ROS may inhibit PDH and KGDH resulting in neuronal and hepatocellular death. Therefore, thiamine or lipoic acid may constitute potential therapeutic agents for Wilson's disease." @default.
• W2064707684 created "2016-06-24" @default.
• W2064707684 creator A5051403739 @default.
• W2064707684 creator A5066110360 @default.
• W2064707684 date "2004-03-21" @default.
• W2064707684 modified "2023-10-17" @default.
• W2064707684 title "Cu2+ toxicity inhibition of mitochondrial dehydrogenases in vitro and in vivo" @default.
• W2064707684 cites W1520164098 @default.
• W2064707684 cites W1528176093 @default.
• W2064707684 cites W1565605186 @default.
• W2064707684 cites W16425910 @default.
• W2064707684 cites W1674124552 @default.
• W2064707684 cites W1821801768 @default.
• W2064707684 cites W1981902498 @default.
• W2064707684 cites W1987594160 @default.
• W2064707684 cites W1989525293 @default.
• W2064707684 cites W1989951299 @default.
• W2064707684 cites W1990004798 @default.
• W2064707684 cites W1994741821 @default.
• W2064707684 cites W1996446520 @default.
• W2064707684 cites W1996579240 @default.
• W2064707684 cites W1997441257 @default.
• W2064707684 cites W2003975298 @default.
• W2064707684 cites W2005462618 @default.
• W2064707684 cites W2005536747 @default.
• W2064707684 cites W2009817860 @default.
• W2064707684 cites W2011681423 @default.
• W2064707684 cites W2012786354 @default.
• W2064707684 cites W2013062925 @default.
• W2064707684 cites W2019170789 @default.
• W2064707684 cites W2019279182 @default.
• W2064707684 cites W2019983337 @default.
• W2064707684 cites W2020324676 @default.
• W2064707684 cites W2022392367 @default.
• W2064707684 cites W2026826823 @default.
• W2064707684 cites W2040793860 @default.
• W2064707684 cites W2044774901 @default.
• W2064707684 cites W2045410354 @default.
• W2064707684 cites W2053712580 @default.
• W2064707684 cites W2059545411 @default.
• W2064707684 cites W2061646790 @default.
• W2064707684 cites W2062834865 @default.
• W2064707684 cites W2063644152 @default.
• W2064707684 cites W2063715310 @default.
• W2064707684 cites W2064570219 @default.
• W2064707684 cites W2070337856 @default.
• W2064707684 cites W2077044335 @default.
• W2064707684 cites W2077703139 @default.
• W2064707684 cites W2078646411 @default.
• W2064707684 cites W2081745487 @default.
• W2064707684 cites W2082872027 @default.
• W2064707684 cites W2088939076 @default.
• W2064707684 cites W2092807733 @default.
• W2064707684 cites W2096386031 @default.
• W2064707684 cites W2105748489 @default.
• W2064707684 cites W2107768418 @default.
• W2064707684 cites W2112633282 @default.
• W2064707684 cites W2119362028 @default.
• W2064707684 cites W2131633241 @default.
• W2064707684 cites W2150742010 @default.
• W2064707684 cites W2174281073 @default.
• W2064707684 cites W70578846 @default.
• W2064707684 doi "https://doi.org/10.1002/ana.20047" @default.
• W2064707684 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/15122704" @default.
• W2064707684 hasPublicationYear "2004" @default.
• W2064707684 type Work @default.
• W2064707684 sameAs 2064707684 @default.
• W2064707684 citedByCount "86" @default.
• W2064707684 countsByYear W20647076842012 @default.
• W2064707684 countsByYear W20647076842013 @default.
• W2064707684 countsByYear W20647076842014 @default.
• W2064707684 countsByYear W20647076842015 @default.
• W2064707684 countsByYear W20647076842016 @default.
• W2064707684 countsByYear W20647076842017 @default.
• W2064707684 countsByYear W20647076842018 @default.
• W2064707684 countsByYear W20647076842019 @default.
• W2064707684 countsByYear W20647076842020 @default.
• W2064707684 countsByYear W20647076842021 @default.
• W2064707684 countsByYear W20647076842022 @default.
• W2064707684 countsByYear W20647076842023 @default.
• W2064707684 crossrefType "journal-article" @default.
• W2064707684 hasAuthorship W2064707684A5051403739 @default.
• W2064707684 hasAuthorship W2064707684A5066110360 @default.
• W2064707684 hasConcept C126093377 @default.
• W2064707684 hasConcept C170493617 @default.
• W2064707684 hasConcept C181199279 @default.
• W2064707684 hasConcept C190098323 @default.
• W2064707684 hasConcept C190283241 @default.
• W2064707684 hasConcept C197957613 @default.
• W2064707684 hasConcept C200886947 @default.
• W2064707684 hasConcept C2776203871 @default.
• W2064707684 hasConcept C2776317432 @default.
• W2064707684 hasConcept C2777202666 @default.
• W2064707684 hasConcept C2778523461 @default.
• W2064707684 hasConcept C2780486903 @default.
• W2064707684 hasConcept C2780912031 @default.
• W2064707684 hasConcept C2781330172 @default.
• W2064707684 hasConcept C28859421 @default.

• next