FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3119464946> ?p ?o ?g. }

• W3119464946 endingPage "103587" @default.
• W3119464946 startingPage "103587" @default.
• W3119464946 abstract "Friedreich's ataxia (FRDA) is a hereditary and predominantly neurodegenerative disease caused by a deficiency of the protein frataxin (FXN). As part of the overall efforts to understand the molecular basis of neurodegeneration in FRDA, a new human neural cell line with doxycycline-induced FXN knockdown was established. This cell line, hereafter referred to as iFKD-SY, is derived from the human neuroblastoma SH-SY5Y and retains the ability to differentiate into mature neuron-like cells. In both proliferating and differentiated iFKD-SY cells, the induction of FXN deficiency is accompanied by increases in oxidative stress and DNA damage, reduced aconitase enzyme activity, higher levels of p53 and p21, activation of caspase-3, and subsequent apoptosis. More interestingly, FXN-deficient iFKD-SY cells exhibit an important transcriptional deregulation in many of the genes implicated in DNA repair pathways. The levels of some crucial proteins involved in DNA repair appear notably diminished. Furthermore, similar changes are found in two additional neural cell models of FXN deficit: primary cultures of FXN-deficient mouse neurons and human olfactory mucosa stem cells obtained from biopsies of FRDA patients. These results suggest that the deficiency of FXN leads to a down-regulation of DNA repair pathways that synergizes with oxidative stress to provoke DNA damage, which may be involved in the pathogenesis of FRDA. Thus, a failure in DNA repair may be considered a shared common molecular mechanism contributing to neurodegeneration in a number of hereditary ataxias including FRDA." @default.
• W3119464946 created "2021-01-18" @default.
• W3119464946 creator A5005858893 @default.
• W3119464946 creator A5040230337 @default.
• W3119464946 creator A5042745492 @default.
• W3119464946 creator A5086981660 @default.
• W3119464946 creator A5087630921 @default.
• W3119464946 date "2021-03-01" @default.
• W3119464946 modified "2023-10-18" @default.
• W3119464946 title "DNA repair pathways are altered in neural cell models of frataxin deficiency" @default.
• W3119464946 cites W1498528035 @default.
• W3119464946 cites W1507242444 @default.
• W3119464946 cites W1745187809 @default.
• W3119464946 cites W1974515171 @default.
• W3119464946 cites W1976396456 @default.
• W3119464946 cites W1978740993 @default.
• W3119464946 cites W1980893206 @default.
• W3119464946 cites W1982276380 @default.
• W3119464946 cites W1983492002 @default.
• W3119464946 cites W1986899307 @default.
• W3119464946 cites W1986989450 @default.
• W3119464946 cites W1993501859 @default.
• W3119464946 cites W1995962173 @default.
• W3119464946 cites W1997826732 @default.
• W3119464946 cites W1998778991 @default.
• W3119464946 cites W2001403270 @default.
• W3119464946 cites W2003753774 @default.
• W3119464946 cites W2008278384 @default.
• W3119464946 cites W2008933430 @default.
• W3119464946 cites W2014034103 @default.
• W3119464946 cites W2014929714 @default.
• W3119464946 cites W2015616869 @default.
• W3119464946 cites W2018530963 @default.
• W3119464946 cites W2020369221 @default.
• W3119464946 cites W2022480988 @default.
• W3119464946 cites W2025541607 @default.
• W3119464946 cites W2025603881 @default.
• W3119464946 cites W2026525843 @default.
• W3119464946 cites W2027945401 @default.
• W3119464946 cites W2032253254 @default.
• W3119464946 cites W2032656149 @default.
• W3119464946 cites W2032730372 @default.
• W3119464946 cites W2035161028 @default.
• W3119464946 cites W2036874681 @default.
• W3119464946 cites W2037606715 @default.
• W3119464946 cites W2040076400 @default.
• W3119464946 cites W2044992022 @default.
• W3119464946 cites W2046694808 @default.
• W3119464946 cites W2048179686 @default.
• W3119464946 cites W2050070465 @default.
• W3119464946 cites W2051265027 @default.
• W3119464946 cites W2052036700 @default.
• W3119464946 cites W2057404038 @default.
• W3119464946 cites W2062604048 @default.
• W3119464946 cites W2063669247 @default.
• W3119464946 cites W2064186088 @default.
• W3119464946 cites W2077274480 @default.
• W3119464946 cites W2079367781 @default.
• W3119464946 cites W2092984629 @default.
• W3119464946 cites W2094198751 @default.
• W3119464946 cites W2100827676 @default.
• W3119464946 cites W2103860452 @default.
• W3119464946 cites W2110864849 @default.
• W3119464946 cites W2110977020 @default.
• W3119464946 cites W2113175203 @default.
• W3119464946 cites W2118572533 @default.
• W3119464946 cites W2121172870 @default.
• W3119464946 cites W2126456755 @default.
• W3119464946 cites W2135020156 @default.
• W3119464946 cites W2135175959 @default.
• W3119464946 cites W2135272501 @default.
• W3119464946 cites W2137107266 @default.
• W3119464946 cites W2138521081 @default.
• W3119464946 cites W2144542859 @default.
• W3119464946 cites W2148732493 @default.
• W3119464946 cites W2152554775 @default.
• W3119464946 cites W2154254146 @default.
• W3119464946 cites W2156827038 @default.
• W3119464946 cites W2158155007 @default.
• W3119464946 cites W2167042539 @default.
• W3119464946 cites W2172149645 @default.
• W3119464946 cites W419853752 @default.
• W3119464946 cites W4248378708 @default.
• W3119464946 doi "https://doi.org/10.1016/j.mcn.2020.103587" @default.
• W3119464946 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/33418083" @default.
• W3119464946 hasPublicationYear "2021" @default.
• W3119464946 type Work @default.
• W3119464946 sameAs 3119464946 @default.
• W3119464946 citedByCount "4" @default.
• W3119464946 countsByYear W31194649462022 @default.
• W3119464946 countsByYear W31194649462023 @default.
• W3119464946 crossrefType "journal-article" @default.
• W3119464946 hasAuthorship W3119464946A5005858893 @default.
• W3119464946 hasAuthorship W3119464946A5040230337 @default.
• W3119464946 hasAuthorship W3119464946A5042745492 @default.
• W3119464946 hasAuthorship W3119464946A5086981660 @default.
• W3119464946 hasAuthorship W3119464946A5087630921 @default.
• W3119464946 hasBestOaLocation W31194649462 @default.

• next