Matches in SemOpenAlex for { <https://semopenalex.org/work/W1561739940> ?p ?o ?g. }
- W1561739940 endingPage "388" @default.
- W1561739940 startingPage "374" @default.
- W1561739940 abstract "The Purkinje cell (PC) degeneration (pcd) phenotype results from mutation in nna1 gene and is associated with the degeneration and death of PCs during the postnatal life. Although the pcd mutation is a model of the ataxic mouse, it shares clinical and pathological characteristics of inherited human spinocerebellar ataxias. PC degeneration in pcd mice provides a useful neuronal system to study nuclear mechanisms involved in DNA damage-dependent neurodegeneration, particularly the contribution of nucleoli and Cajal bodies (CBs). Both nuclear structures are engaged in housekeeping functions for neuronal survival, the biogenesis of ribosomes and the maturation of snRNPs and snoRNPs required for pre-mRNA and pre-rRNA processing, respectively. In this study, we use ultrastructural analysis, in situ transcription assay and molecular markers for DNA damage, nucleoli and CB components to demonstrate that PC degeneration involves the progressive accumulation of nuclear DNA damage associated with disruption of nucleoli and CBs, disassembly of polyribosomes into monoribosomes, ribophagy and shut down of nucleolar and extranucleolar transcription. Microarray analysis reveals that four genes encoding repressors of nucleolar rRNA synthesis (p53, Rb, PTEN and SNF2) are upregulated in the cerebellum of pcd mice. Collectively, these data support that nucleolar and CB alterations are hallmarks of DNA damage-induced neurodegeneration." @default.
- W1561739940 created "2016-06-24" @default.
- W1561739940 creator A5021395949 @default.
- W1561739940 creator A5038051768 @default.
- W1561739940 creator A5043414758 @default.
- W1561739940 creator A5062372221 @default.
- W1561739940 creator A5074024292 @default.
- W1561739940 creator A5089958998 @default.
- W1561739940 date "2010-11-30" @default.
- W1561739940 modified "2023-09-30" @default.
- W1561739940 title "Nucleolar Disruption and Cajal Body Disassembly are Nuclear Hallmarks of DNA Damage-Induced Neurodegeneration in Purkinje Cells" @default.
- W1561739940 cites W1497091656 @default.
- W1561739940 cites W1520808973 @default.
- W1561739940 cites W1536477417 @default.
- W1561739940 cites W1580906825 @default.
- W1561739940 cites W1653723521 @default.
- W1561739940 cites W1965071443 @default.
- W1561739940 cites W1968665479 @default.
- W1561739940 cites W1969521521 @default.
- W1561739940 cites W1970315917 @default.
- W1561739940 cites W1973026541 @default.
- W1561739940 cites W1973309606 @default.
- W1561739940 cites W1975431812 @default.
- W1561739940 cites W1976276205 @default.
- W1561739940 cites W1979193782 @default.
- W1561739940 cites W1982447331 @default.
- W1561739940 cites W1983811755 @default.
- W1561739940 cites W1985541369 @default.
- W1561739940 cites W1987687980 @default.
- W1561739940 cites W1988869829 @default.
- W1561739940 cites W1989684700 @default.
- W1561739940 cites W1995178146 @default.
- W1561739940 cites W1999446997 @default.
- W1561739940 cites W2000443400 @default.
- W1561739940 cites W2002373110 @default.
- W1561739940 cites W2005664674 @default.
- W1561739940 cites W2006273621 @default.
- W1561739940 cites W2012674840 @default.
- W1561739940 cites W2019528668 @default.
- W1561739940 cites W2019835154 @default.
- W1561739940 cites W2027047074 @default.
- W1561739940 cites W2027085235 @default.
- W1561739940 cites W2034418403 @default.
- W1561739940 cites W2035244376 @default.
- W1561739940 cites W2035550923 @default.
- W1561739940 cites W2036356789 @default.
- W1561739940 cites W2040196647 @default.
- W1561739940 cites W2052036700 @default.
- W1561739940 cites W2056153549 @default.
- W1561739940 cites W2057400223 @default.
- W1561739940 cites W2063501009 @default.
- W1561739940 cites W2064122880 @default.
- W1561739940 cites W2066318904 @default.
- W1561739940 cites W2067574987 @default.
- W1561739940 cites W2076826746 @default.
- W1561739940 cites W2082810375 @default.
- W1561739940 cites W2086386368 @default.
- W1561739940 cites W2090522280 @default.
- W1561739940 cites W2091345758 @default.
- W1561739940 cites W2091927985 @default.
- W1561739940 cites W2092869829 @default.
- W1561739940 cites W2098278518 @default.
- W1561739940 cites W2104746705 @default.
- W1561739940 cites W2118107253 @default.
- W1561739940 cites W2128680272 @default.
- W1561739940 cites W2131418908 @default.
- W1561739940 cites W2136367337 @default.
- W1561739940 cites W2136513747 @default.
- W1561739940 cites W2136575970 @default.
- W1561739940 cites W2138024897 @default.
- W1561739940 cites W2138165292 @default.
- W1561739940 cites W2142934782 @default.
- W1561739940 cites W2143079500 @default.
- W1561739940 cites W2146842824 @default.
- W1561739940 cites W2152606927 @default.
- W1561739940 cites W2153507013 @default.
- W1561739940 cites W2158452003 @default.
- W1561739940 cites W2161902637 @default.
- W1561739940 cites W2161972131 @default.
- W1561739940 cites W2169905889 @default.
- W1561739940 cites W2170989872 @default.
- W1561739940 cites W2185201703 @default.
- W1561739940 cites W4245552230 @default.
- W1561739940 doi "https://doi.org/10.1111/j.1750-3639.2010.00461.x" @default.
- W1561739940 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/8094285" @default.
- W1561739940 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/21054627" @default.
- W1561739940 hasPublicationYear "2010" @default.
- W1561739940 type Work @default.
- W1561739940 sameAs 1561739940 @default.
- W1561739940 citedByCount "56" @default.
- W1561739940 countsByYear W15617399402012 @default.
- W1561739940 countsByYear W15617399402013 @default.
- W1561739940 countsByYear W15617399402014 @default.
- W1561739940 countsByYear W15617399402015 @default.
- W1561739940 countsByYear W15617399402016 @default.
- W1561739940 countsByYear W15617399402017 @default.
- W1561739940 countsByYear W15617399402018 @default.
- W1561739940 countsByYear W15617399402019 @default.