FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3008172210> ?p ?o ?g. }

• W3008172210 abstract "Summary Although the intricate and prolonged development of the human brain critically distinguishes it from other mammals 1 , our current understanding of neurodevelopmental diseases is largely based on work using animal models. Recent studies revealed that neural progenitors in the human brain are profoundly different from those found in rodent animal models 2–5 . Moreover, post-mortem studies revealed extensive migration of interneurons into the late-gestational and post-natal human prefrontal cortex that does not occur in rodents 6 . Here, we use cerebral organoids to show that overproduction of mid-gestational human interneurons causes Tuberous Sclerosis Complex (TSC), a severe neuro-developmental disorder associated with mutations in TSC1 and TSC2 . We identify a previously uncharacterized population of caudal late interneuron progenitors, the CLIP-cells. In organoids derived from patients carrying heterozygous TSC2 mutations, dysregulation of mTOR signaling leads to CLIP-cell over-proliferation and formation of cortical tubers and subependymal tumors. Surprisingly, second-hit events resulting from copy-neutral loss-of-heterozygosity (cnLOH) are not causative for but occur during the progression of tumor lesions. Instead, EGFR signaling is required for tumor proliferation, opening up a promising approach to treat TSC lesions. Our study demonstrates that the analysis of developmental disorders in organoid models can lead to fundamental insights into human brain development and neuropsychiatric disorders." @default.
• W3008172210 created "2020-03-06" @default.
• W3008172210 creator A5008803187 @default.
• W3008172210 creator A5014790032 @default.
• W3008172210 creator A5017540861 @default.
• W3008172210 creator A5023139971 @default.
• W3008172210 creator A5038622666 @default.
• W3008172210 creator A5039689620 @default.
• W3008172210 creator A5047746348 @default.
• W3008172210 creator A5052752045 @default.
• W3008172210 creator A5067401650 @default.
• W3008172210 creator A5076983676 @default.
• W3008172210 creator A5077522618 @default.
• W3008172210 date "2020-02-27" @default.
• W3008172210 modified "2023-10-15" @default.
• W3008172210 title "Cerebral organoid model reveals excessive proliferation of human caudal late interneuron progenitors in Tuberous Sclerosis Complex" @default.
• W3008172210 cites W1486174444 @default.
• W3008172210 cites W156748014 @default.
• W3008172210 cites W1874466506 @default.
• W3008172210 cites W1899552890 @default.
• W3008172210 cites W1970212739 @default.
• W3008172210 cites W1973632589 @default.
• W3008172210 cites W1975978177 @default.
• W3008172210 cites W1985958520 @default.
• W3008172210 cites W2000345419 @default.
• W3008172210 cites W2003707718 @default.
• W3008172210 cites W2010994321 @default.
• W3008172210 cites W2013258896 @default.
• W3008172210 cites W2018286352 @default.
• W3008172210 cites W2020209656 @default.
• W3008172210 cites W2032555040 @default.
• W3008172210 cites W2035495486 @default.
• W3008172210 cites W2045435533 @default.
• W3008172210 cites W2050790870 @default.
• W3008172210 cites W2051364266 @default.
• W3008172210 cites W2056084345 @default.
• W3008172210 cites W2070667172 @default.
• W3008172210 cites W2076268719 @default.
• W3008172210 cites W2077858999 @default.
• W3008172210 cites W2087441158 @default.
• W3008172210 cites W2087531974 @default.
• W3008172210 cites W2100428079 @default.
• W3008172210 cites W2106253915 @default.
• W3008172210 cites W2137384928 @default.
• W3008172210 cites W2139486959 @default.
• W3008172210 cites W2141091417 @default.
• W3008172210 cites W2155018172 @default.
• W3008172210 cites W2165121719 @default.
• W3008172210 cites W2165216871 @default.
• W3008172210 cites W2169786878 @default.
• W3008172210 cites W2461453397 @default.
• W3008172210 cites W2529762510 @default.
• W3008172210 cites W2546476155 @default.
• W3008172210 cites W2553053943 @default.
• W3008172210 cites W2578839378 @default.
• W3008172210 cites W2588142332 @default.
• W3008172210 cites W2588870577 @default.
• W3008172210 cites W2607713745 @default.
• W3008172210 cites W2617770907 @default.
• W3008172210 cites W2626329567 @default.
• W3008172210 cites W2772624269 @default.
• W3008172210 cites W2781205255 @default.
• W3008172210 cites W2782793342 @default.
• W3008172210 cites W2804960675 @default.
• W3008172210 cites W2883445821 @default.
• W3008172210 cites W2886007983 @default.
• W3008172210 cites W2901180214 @default.
• W3008172210 cites W2905157186 @default.
• W3008172210 cites W2949020598 @default.
• W3008172210 cites W2952000543 @default.
• W3008172210 cites W2969948459 @default.
• W3008172210 cites W3004345103 @default.
• W3008172210 cites W4210953366 @default.
• W3008172210 cites W4252335603 @default.
• W3008172210 doi "https://doi.org/10.1101/2020.02.27.967802" @default.
• W3008172210 hasPublicationYear "2020" @default.
• W3008172210 type Work @default.
• W3008172210 sameAs 3008172210 @default.
• W3008172210 citedByCount "5" @default.
• W3008172210 countsByYear W30081722102020 @default.
• W3008172210 countsByYear W30081722102021 @default.
• W3008172210 countsByYear W30081722102022 @default.
• W3008172210 crossrefType "posted-content" @default.
• W3008172210 hasAuthorship W3008172210A5008803187 @default.
• W3008172210 hasAuthorship W3008172210A5014790032 @default.
• W3008172210 hasAuthorship W3008172210A5017540861 @default.
• W3008172210 hasAuthorship W3008172210A5023139971 @default.
• W3008172210 hasAuthorship W3008172210A5038622666 @default.
• W3008172210 hasAuthorship W3008172210A5039689620 @default.
• W3008172210 hasAuthorship W3008172210A5047746348 @default.
• W3008172210 hasAuthorship W3008172210A5052752045 @default.
• W3008172210 hasAuthorship W3008172210A5067401650 @default.
• W3008172210 hasAuthorship W3008172210A5076983676 @default.
• W3008172210 hasAuthorship W3008172210A5077522618 @default.
• W3008172210 hasBestOaLocation W30081722101 @default.
• W3008172210 hasConcept C136834591 @default.
• W3008172210 hasConcept C142724271 @default.
• W3008172210 hasConcept C169760540 @default.
• W3008172210 hasConcept C17077164 @default.
• W3008172210 hasConcept C201750760 @default.

• next