FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2070086306> ?p ?o ?g. }

• W2070086306 endingPage "3863" @default.
• W2070086306 startingPage "3858" @default.
• W2070086306 abstract "Neuronal cytoplasmic and intranuclear aggregates of RNA-binding protein TDP-43 are a hallmark feature of neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). ALS and FTLD show a considerable clinical and pathological overlap and occur as both familial and sporadic forms. Though missense mutations in TDP-43 cause rare forms of familial ALS, it is not yet known whether this is due to loss of TDP-43 function or gain of aberrant function. Moreover, the role of wild-type (WT) TDP-43, associated with the majority of familial and sporadic ALS/FTLD patients, is also currently unknown. Generating homozygous and hemizygous WT human TDP-43 transgenic mouse lines, we show here a dose-dependent degeneration of cortical and spinal motor neurons and development of spastic quadriplegia reminiscent of ALS. A dose-dependent degeneration of nonmotor cortical and subcortical neurons characteristic of FTLD was also observed. Neurons in the affected spinal cord and brain regions showed accumulation of TDP-43 nuclear and cytoplasmic aggregates that were both ubiquitinated and phosphorylated as observed in ALS/FTLD patients. Moreover, the characteristic ≈25-kDa C-terminal fragments (CTFs) were also recovered from nuclear fractions and correlated with disease development and progression in WT TDP-43 mice. These findings suggest that ≈25-kDa TDP-43 CTFs are noxious to neurons by a gain of aberrant nuclear function." @default.
• W2070086306 created "2016-06-24" @default.
• W2070086306 creator A5003895499 @default.
• W2070086306 creator A5008355134 @default.
• W2070086306 creator A5031871316 @default.
• W2070086306 creator A5034537815 @default.
• W2070086306 creator A5039639102 @default.
• W2070086306 creator A5046649201 @default.
• W2070086306 creator A5057641888 @default.
• W2070086306 creator A5058816295 @default.
• W2070086306 creator A5085805590 @default.
• W2070086306 creator A5085953981 @default.
• W2070086306 date "2010-02-23" @default.
• W2070086306 modified "2023-10-18" @default.
• W2070086306 title "TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration" @default.
• W2070086306 cites W1619082318 @default.
• W2070086306 cites W1966682365 @default.
• W2070086306 cites W1971323868 @default.
• W2070086306 cites W1974298458 @default.
• W2070086306 cites W1975860260 @default.
• W2070086306 cites W1977399503 @default.
• W2070086306 cites W1980825524 @default.
• W2070086306 cites W1980907693 @default.
• W2070086306 cites W1984145170 @default.
• W2070086306 cites W1991611976 @default.
• W2070086306 cites W1996431592 @default.
• W2070086306 cites W2001093038 @default.
• W2070086306 cites W2003618511 @default.
• W2070086306 cites W2006897215 @default.
• W2070086306 cites W2010240177 @default.
• W2070086306 cites W2019574244 @default.
• W2070086306 cites W2022367303 @default.
• W2070086306 cites W2025122795 @default.
• W2070086306 cites W2026282890 @default.
• W2070086306 cites W2027720028 @default.
• W2070086306 cites W2029374248 @default.
• W2070086306 cites W2031964319 @default.
• W2070086306 cites W2041451000 @default.
• W2070086306 cites W2045189928 @default.
• W2070086306 cites W2060076138 @default.
• W2070086306 cites W2064850602 @default.
• W2070086306 cites W2066952414 @default.
• W2070086306 cites W2071991588 @default.
• W2070086306 cites W2072413533 @default.
• W2070086306 cites W2076631319 @default.
• W2070086306 cites W2080861984 @default.
• W2070086306 cites W2084394003 @default.
• W2070086306 cites W2084561728 @default.
• W2070086306 cites W2091145925 @default.
• W2070086306 cites W2091547575 @default.
• W2070086306 cites W2096410114 @default.
• W2070086306 cites W2100111801 @default.
• W2070086306 cites W2104658204 @default.
• W2070086306 cites W2107768418 @default.
• W2070086306 cites W2116086426 @default.
• W2070086306 cites W2120180072 @default.
• W2070086306 cites W2123491442 @default.
• W2070086306 cites W2131725903 @default.
• W2070086306 cites W2134051267 @default.
• W2070086306 cites W2137048103 @default.
• W2070086306 cites W2152098733 @default.
• W2070086306 cites W2322852998 @default.
• W2070086306 doi "https://doi.org/10.1073/pnas.0912417107" @default.
• W2070086306 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2840518" @default.
• W2070086306 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20133711" @default.
• W2070086306 hasPublicationYear "2010" @default.
• W2070086306 type Work @default.
• W2070086306 sameAs 2070086306 @default.
• W2070086306 citedByCount "486" @default.
• W2070086306 countsByYear W20700863062012 @default.
• W2070086306 countsByYear W20700863062013 @default.
• W2070086306 countsByYear W20700863062014 @default.
• W2070086306 countsByYear W20700863062015 @default.
• W2070086306 countsByYear W20700863062016 @default.
• W2070086306 countsByYear W20700863062017 @default.
• W2070086306 countsByYear W20700863062018 @default.
• W2070086306 countsByYear W20700863062019 @default.
• W2070086306 countsByYear W20700863062020 @default.
• W2070086306 countsByYear W20700863062021 @default.
• W2070086306 countsByYear W20700863062022 @default.
• W2070086306 countsByYear W20700863062023 @default.
• W2070086306 crossrefType "journal-article" @default.
• W2070086306 hasAuthorship W2070086306A5003895499 @default.
• W2070086306 hasAuthorship W2070086306A5008355134 @default.
• W2070086306 hasAuthorship W2070086306A5031871316 @default.
• W2070086306 hasAuthorship W2070086306A5034537815 @default.
• W2070086306 hasAuthorship W2070086306A5039639102 @default.
• W2070086306 hasAuthorship W2070086306A5046649201 @default.
• W2070086306 hasAuthorship W2070086306A5057641888 @default.
• W2070086306 hasAuthorship W2070086306A5058816295 @default.
• W2070086306 hasAuthorship W2070086306A5085805590 @default.
• W2070086306 hasAuthorship W2070086306A5085953981 @default.
• W2070086306 hasBestOaLocation W20700863062 @default.
• W2070086306 hasConcept C102230213 @default.
• W2070086306 hasConcept C104317684 @default.
• W2070086306 hasConcept C141035611 @default.
• W2070086306 hasConcept C142724271 @default.
• W2070086306 hasConcept C169760540 @default.

• next