FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W1977510154> ?p ?o ?g. }

• W1977510154 endingPage "179" @default.
• W1977510154 startingPage "172" @default.
• W1977510154 abstract "Leber's hereditary optic neuropathy (LHON) was the first maternally inherited disease to be associated with point mutations in mitochondrial DNA and is now considered the most prevalent mitochondrial disorder. The pathology is characterized by selective loss of ganglion cells in the retina leading to central vision loss and optic atrophy, prevalently in young males. The pathogenic mtDNA point mutations for LHON affect complex I with the double effect of lowering the ATP synthesis driven by complex I substrates and increasing oxidative stress chronically. In this review, we first consider the biochemical changes associated with the proton-translocating NADH-quinone oxidoreductase of mitochondria in cybrid cells carrying the most common LHON mutations. However, the LHON cybrid bioenergetic dysfunction is essentially compensated under normal conditions, i.e. in glucose medium, but is unrevealed by stressful conditions such as growing cybrids in glucose free/galactose medium, which forces cells to rely only on respiratory chain for ATP synthesis. In fact, the second part of this review deals with the investigation of LHON cybrid death pathway in galactose medium. The parallel marked changes in antioxidant enzymes, during the time-course of galactose experiments, also reveal a relevant role played by oxidative stress. The LHON cybrid model sheds light on the complex interplay amongst the different levels of biochemical consequences deriving from complex I mutations in determining neurodegeneration in LHON, and suggests an unsuspected role of bioenergetics in shaping cell death pathways." @default.
• W1977510154 created "2016-06-24" @default.
• W1977510154 creator A5001687552 @default.
• W1977510154 creator A5008008546 @default.
• W1977510154 creator A5023379236 @default.
• W1977510154 creator A5045919146 @default.
• W1977510154 creator A5052548286 @default.
• W1977510154 creator A5057433492 @default.
• W1977510154 creator A5064357836 @default.
• W1977510154 creator A5065278268 @default.
• W1977510154 creator A5070622522 @default.
• W1977510154 date "2004-07-01" @default.
• W1977510154 modified "2023-10-01" @default.
• W1977510154 title "Bioenergetics shapes cellular death pathways in Leber's hereditary optic neuropathy: a model of mitochondrial neurodegeneration" @default.
• W1977510154 cites W1480948328 @default.
• W1977510154 cites W1530991954 @default.
• W1977510154 cites W1845459216 @default.
• W1977510154 cites W1967304767 @default.
• W1977510154 cites W1971140390 @default.
• W1977510154 cites W1976628426 @default.
• W1977510154 cites W1980681204 @default.
• W1977510154 cites W1997640580 @default.
• W1977510154 cites W2000789495 @default.
• W1977510154 cites W2001083394 @default.
• W1977510154 cites W2010741329 @default.
• W1977510154 cites W2015808639 @default.
• W1977510154 cites W2016163301 @default.
• W1977510154 cites W2017347785 @default.
• W1977510154 cites W2018417798 @default.
• W1977510154 cites W2019319567 @default.
• W1977510154 cites W2024973030 @default.
• W1977510154 cites W2034915619 @default.
• W1977510154 cites W2037817877 @default.
• W1977510154 cites W2038055601 @default.
• W1977510154 cites W2039162492 @default.
• W1977510154 cites W2040636296 @default.
• W1977510154 cites W2043165883 @default.
• W1977510154 cites W2044417730 @default.
• W1977510154 cites W2046307510 @default.
• W1977510154 cites W2050057361 @default.
• W1977510154 cites W2055595633 @default.
• W1977510154 cites W2058837644 @default.
• W1977510154 cites W2065629099 @default.
• W1977510154 cites W2067124266 @default.
• W1977510154 cites W2071888455 @default.
• W1977510154 cites W2079904655 @default.
• W1977510154 cites W2083612483 @default.
• W1977510154 cites W2088658333 @default.
• W1977510154 cites W2093325321 @default.
• W1977510154 cites W2097466887 @default.
• W1977510154 cites W2107396020 @default.
• W1977510154 cites W2118464854 @default.
• W1977510154 cites W2119683782 @default.
• W1977510154 cites W2120639051 @default.
• W1977510154 cites W2121147116 @default.
• W1977510154 cites W2138993325 @default.
• W1977510154 cites W2141981416 @default.
• W1977510154 cites W2144675621 @default.
• W1977510154 cites W2144701362 @default.
• W1977510154 cites W2153769207 @default.
• W1977510154 cites W2159650080 @default.
• W1977510154 cites W2163370539 @default.
• W1977510154 cites W2164359443 @default.
• W1977510154 cites W2171440342 @default.
• W1977510154 cites W2171715833 @default.
• W1977510154 cites W2462327257 @default.
• W1977510154 doi "https://doi.org/10.1016/j.bbabio.2004.05.009" @default.
• W1977510154 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/15282189" @default.
• W1977510154 hasPublicationYear "2004" @default.
• W1977510154 type Work @default.
• W1977510154 sameAs 1977510154 @default.
• W1977510154 citedByCount "110" @default.
• W1977510154 countsByYear W19775101542012 @default.
• W1977510154 countsByYear W19775101542013 @default.
• W1977510154 countsByYear W19775101542014 @default.
• W1977510154 countsByYear W19775101542015 @default.
• W1977510154 countsByYear W19775101542016 @default.
• W1977510154 countsByYear W19775101542017 @default.
• W1977510154 countsByYear W19775101542018 @default.
• W1977510154 countsByYear W19775101542019 @default.
• W1977510154 countsByYear W19775101542020 @default.
• W1977510154 countsByYear W19775101542021 @default.
• W1977510154 countsByYear W19775101542022 @default.
• W1977510154 countsByYear W19775101542023 @default.
• W1977510154 crossrefType "journal-article" @default.
• W1977510154 hasAuthorship W1977510154A5001687552 @default.
• W1977510154 hasAuthorship W1977510154A5008008546 @default.
• W1977510154 hasAuthorship W1977510154A5023379236 @default.
• W1977510154 hasAuthorship W1977510154A5045919146 @default.
• W1977510154 hasAuthorship W1977510154A5052548286 @default.
• W1977510154 hasAuthorship W1977510154A5057433492 @default.
• W1977510154 hasAuthorship W1977510154A5064357836 @default.
• W1977510154 hasAuthorship W1977510154A5065278268 @default.
• W1977510154 hasAuthorship W1977510154A5070622522 @default.
• W1977510154 hasConcept C100206155 @default.
• W1977510154 hasConcept C104317684 @default.
• W1977510154 hasConcept C142724271 @default.
• W1977510154 hasConcept C169760540 @default.

• next