Smoothened-antagonists reverse homogentisic acid-induced alterations of Hedgehog signaling and primary cilium length in alkaptonuria
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چکیده
منابع مشابه
Primary Cilium Patched 1 Regulates Hedgehog Signaling
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The seven-transmembrane receptor Smoothened (Smo) activates all Hedgehog (Hh) signaling by translocation into the primary cilia (PC), but how this is regulated is not well understood. Here we show that Pitchfork (Pifo) and the G protein-coupled receptor associated sorting protein 2 (Gprasp2) are essential components of an Hh induced ciliary targeting complex able to regulate Smo translocation t...
متن کاملZfp423 Regulates Sonic Hedgehog Signaling via Primary Cilium Function
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, l...
متن کاملPatched1 regulates hedgehog signaling at the primary cilium.
Primary cilia are essential for transduction of the Hedgehog (Hh) signal in mammals. We investigated the role of primary cilia in regulation of Patched1 (Ptc1), the receptor for Sonic Hedgehog (Shh). Ptc1 localized to cilia and inhibited Smoothened (Smo) by preventing its accumulation within cilia. When Shh bound to Ptc1, Ptc1 left the cilia, leading to accumulation of Smo and activation of sig...
متن کاملRohatgi Patched 1 Regulates Hedgehog Signaling at the Primary Cilium
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ژورنال
عنوان ژورنال: Journal of Cellular Physiology
سال: 2017
ISSN: 0021-9541
DOI: 10.1002/jcp.25761