FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2949915089> ?p ?o ?g. }

• W2949915089 endingPage "e1006357" @default.
• W2949915089 startingPage "e1006357" @default.
• W2949915089 abstract "Zfp423 encodes a 30-zinc finger transcription factor that intersects several canonical signaling pathways. Zfp423 mutations result in ciliopathy-related phenotypes, including agenesis of the cerebellar vermis in mice and Joubert syndrome (JBTS19) and nephronophthisis (NPHP14) in humans. Unlike most ciliopathy genes, Zfp423 encodes a nuclear protein and its developmental expression is complex, leading to alternative proposals for cellular mechanisms. Here we show that Zfp423 is expressed by cerebellar granule cell precursors, that loss of Zfp423 in these precursors leads to cell-intrinsic reduction in proliferation, loss of response to Shh, and primary cilia abnormalities that include diminished frequency of both Smoothened and IFT88 localization. Loss of Zfp423 alters expression of several genes encoding key cilium components, including increased expression of Tulp3. Tulp3 is a direct binding target of Zfp423 and reducing the overexpression of Tulp3 in Zfp423-deficient cells suppresses Smoothened translocation defects. These results define Zfp423 deficiency as a bona fide ciliopathy, acting upstream of Shh signaling, and indicate a mechanism intrinsic to granule cell precursors for the resulting cerebellar hypoplasia." @default.
• W2949915089 created "2019-06-27" @default.
• W2949915089 creator A5038813737 @default.
• W2949915089 creator A5059554334 @default.
• W2949915089 date "2016-10-11" @default.
• W2949915089 modified "2023-09-25" @default.
• W2949915089 title "Zfp423 Regulates Sonic Hedgehog Signaling via Primary Cilium Function" @default.
• W2949915089 cites W142203824 @default.
• W2949915089 cites W1845077221 @default.
• W2949915089 cites W1966755445 @default.
• W2949915089 cites W1967290608 @default.
• W2949915089 cites W1967977447 @default.
• W2949915089 cites W1968251266 @default.
• W2949915089 cites W1971060771 @default.
• W2949915089 cites W1985719373 @default.
• W2949915089 cites W1986595328 @default.
• W2949915089 cites W1991915230 @default.
• W2949915089 cites W1993465232 @default.
• W2949915089 cites W1998796212 @default.
• W2949915089 cites W2001170778 @default.
• W2949915089 cites W2001862736 @default.
• W2949915089 cites W2006399184 @default.
• W2949915089 cites W2014677321 @default.
• W2949915089 cites W2018170841 @default.
• W2949915089 cites W2018911042 @default.
• W2949915089 cites W2021691433 @default.
• W2949915089 cites W2021880506 @default.
• W2949915089 cites W2022160583 @default.
• W2949915089 cites W2023040415 @default.
• W2949915089 cites W2026137500 @default.
• W2949915089 cites W2028417396 @default.
• W2949915089 cites W2032792789 @default.
• W2949915089 cites W2036582717 @default.
• W2949915089 cites W2037700340 @default.
• W2949915089 cites W2039238855 @default.
• W2949915089 cites W2042657989 @default.
• W2949915089 cites W2051301625 @default.
• W2949915089 cites W2055211442 @default.
• W2949915089 cites W2055884152 @default.
• W2949915089 cites W2064462911 @default.
• W2949915089 cites W2064959533 @default.
• W2949915089 cites W2066672792 @default.
• W2949915089 cites W2067933362 @default.
• W2949915089 cites W2072547641 @default.
• W2949915089 cites W2074814691 @default.
• W2949915089 cites W2079619073 @default.
• W2949915089 cites W2083988049 @default.
• W2949915089 cites W2085356690 @default.
• W2949915089 cites W2089766172 @default.
• W2949915089 cites W2091684557 @default.
• W2949915089 cites W2094758282 @default.
• W2949915089 cites W2094838109 @default.
• W2949915089 cites W2096049380 @default.
• W2949915089 cites W2096155765 @default.
• W2949915089 cites W2098287644 @default.
• W2949915089 cites W2100793213 @default.
• W2949915089 cites W2103344845 @default.
• W2949915089 cites W2105663489 @default.
• W2949915089 cites W2106974809 @default.
• W2949915089 cites W2114104545 @default.
• W2949915089 cites W2115235864 @default.
• W2949915089 cites W2117492061 @default.
• W2949915089 cites W2117690421 @default.
• W2949915089 cites W2118366155 @default.
• W2949915089 cites W2122160273 @default.
• W2949915089 cites W2123106337 @default.
• W2949915089 cites W2123700457 @default.
• W2949915089 cites W2124070731 @default.
• W2949915089 cites W2127882408 @default.
• W2949915089 cites W2128801071 @default.
• W2949915089 cites W2130410032 @default.
• W2949915089 cites W2135979384 @default.
• W2949915089 cites W2136634228 @default.
• W2949915089 cites W2137279272 @default.
• W2949915089 cites W2138069365 @default.
• W2949915089 cites W2138779444 @default.
• W2949915089 cites W2140864413 @default.
• W2949915089 cites W2142673763 @default.
• W2949915089 cites W2148031208 @default.
• W2949915089 cites W2152045787 @default.
• W2949915089 cites W2152523782 @default.
• W2949915089 cites W2153260902 @default.
• W2949915089 cites W2155768517 @default.
• W2949915089 cites W2159718837 @default.
• W2949915089 cites W2160084509 @default.
• W2949915089 cites W2169456326 @default.
• W2949915089 cites W2170193319 @default.
• W2949915089 cites W2291982250 @default.
• W2949915089 cites W2312772969 @default.
• W2949915089 cites W2409470013 @default.
• W2949915089 cites W4211232356 @default.
• W2949915089 doi "https://doi.org/10.1371/journal.pgen.1006357" @default.
• W2949915089 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5065120" @default.
• W2949915089 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27727273" @default.
• W2949915089 hasPublicationYear "2016" @default.
• W2949915089 type Work @default.
• W2949915089 sameAs 2949915089 @default.
• W2949915089 citedByCount "27" @default.

• next