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data illustrate that persistent Hh signaling in the palatal epithelium contributes to the etiology and pathogenesis of submucous cleft palate through its interaction with a p63 (zeige CKAP4 Proteine)/Irf6 (zeige IRF6 Proteine)-dependent biological regulatory loop and through a p63 (zeige CKAP4 Proteine)-induced cell adhesion network.
SOX2 (zeige SOX2 Proteine) functions downstream of HH signaling to regulate lingual epithelium homeostasis.
High SHH expression is associated with medulloblastoma formation.
Identify SMO-dependent Shh signalling as a specific process for the activation of adventitial fibroblasts and the subsequent proliferation of smooth muscle cells and neointima formation.
In the mutant Hammer toe (Hm) genome, a 150-kb noncoding DNA fragment from chromosome 14 is inserted into the region upstream of the Sonic hedgehog (Shh) promoter in chromosome 5. Two different regions are necessary for the syndactyly phenotype of Hm.
Data show that Sonic hedgehog (Shh), which encodes a secreted protein signal, is expressed in the sensory neurons, and that experimental ablation of neuronal Shh expression causes loss of taste receptor cells (TRCs).
Here by inducing expression of constitutively active Smoothened (SmoM2) or Gli2 (DeltaNGli2) in the adipocyte lineage of postnatal mice, the authors show that targeted activation of Hedgehog signaling suppresses high-fat-diet-induced obesity and improves whole-body glucose tolerance and insulin (zeige INS Proteine) sensitivity.
These data suggest a potential novel mechanism in which alterations in SHH variance during evolution may have driven changes in limb patterning and digit length.
Epithelial-mesenchymal transition programs promote basal mammary stem cell and tumor-initiating cell stemness by inducing primary ciliogenesis and Hedgehog signaling.
the knockdown of Ihh (zeige IHH Proteine) suppressed osteoblast growth and differentiation via a mechanism which may be associated with the TGF-beta (zeige TGFB1 Proteine)/Smad (zeige SMAD1 Proteine) and OPG/RANKL (zeige TNFSF11 Proteine) signaling pathways.
SHH-related signaling pathway affects antineoplastic drug resistance in cultured glioma cells.
SHH is expressed in cilia in the airway epithelial cells.SHH may mediate noncanonical hedgehog signaling through motile cilia to dampen respiratory defenses.
High SHH expression is associated with radioresistance in esophageal adenocarcinoma.
In conclusion, our data suggest that overexpression of the Hedgehog components SHH, GLI2 and FOXA2 (zeige FOXA2 Proteine) might be used as markers of an aggressive hemangioma.
These findings showed the upexpression of sonic hedgehog and vascular endothelial growth factor (zeige VEGF Proteine) with co-localization in varicocele veins which imply that the reducing hypoxia or using sonic hedgehog antagonists may be helpful for this vascular disease.
Shh and Gli1 (zeige GLI1 Proteine) expression were associated with lymph node metastasis, TNM (zeige ODZ1 Proteine) stage and tumor recurrence, suggesting Shh and Gli1 (zeige GLI1 Proteine) protein could become the valuable biomarker in evaluating the lymph node metastasis in oral squamous cell carcinoma.
Case Report: medullablastoma with activated SHH expression.
NAFLD progression is usually accompanied by activation of the Sonic hedgehog (SHH) pathway leading to fibrous buildup (scar tissue) and inflammation of the liver tissue. For the first time patients with holoprosencephaly, a disease caused by SHH signaling mutations, are shown to have increased liver steatosis independent of obesity.
SHH-dependent E-ligase Midline1 (zeige MID1 Proteine) regulates ubiquitin-mediated proteasomal degradation of Pax6 (zeige PAX6 Proteine) during visual system development.
Data indicate that sonic hedgehog is expressed exclusively in the notochord but not in the spinal cord of the regenerate.
Dzip1 (zeige DZIP1 Proteine)-dependent stabilization of Spop (zeige SPOP Proteine)/HIB is evolutionarily conserved and essential for proper regulation of Gli (zeige GLI1 Proteine)/Ci proteins in the Hh pathway.
Notch (zeige NOTCH1 Proteine) signaling promotes floor plate and hypochord fates over notochord, but has variable effects on Shh expression in the midline.
These results indicate that electrical activity and second-messenger signaling mediate Shh action in embryonic spinal neurons.
Connective tissue-specific expression of BMP-4 (zeige BMP4 Proteine) mRNA is up-regulated by sonic hedgehog.
In an examination of signaling pathways in developing Xenopus lung, shh but not ihh (zeige IHH Proteine) was expressed in the very anterior part of early lung epithelium.
Data propose that Shh serves as a ventral optic tract repellent that helps to define the caudal boundary for retinal axons in the diencephalon, and that this signaling is also required for initial target recognition at the optic tectum.
The forebrain of Xenopus revealed a largely conserved pattern of Shh expression among tetrapods.
Hedgehog regulates superficial slow muscle fibres in Xenopus and tetrapod trunk myogenesis
study shows evolutionary alteration of a Ptch1 (zeige PTCH1 Proteine) cis (zeige CISH Proteine)-regulatory module, which no longer responds to graded SHH signalling during bovine handplate development
Mutation of hmgcs1 (zeige HMGCS1 Proteine) had no effect on Shh signaling at 2 and 3 days post fertilization (dpf), but did result in a decrease in the expression of gli1 (zeige GLI1 Proteine), a known Shh target gene, at 4 dpf, after morphological deficits in craniofacial development and chondrocyte differentiation were observed in hmgcs1 (zeige HMGCS1 Proteine) mutants.
Shha/Smo is functionally dedicated to ray branching during fin regeneration.
Shh and Rx3 govern formation of a distinct progenitor domain that elaborates patterning through its anisotropic growth and differentiation.
Time-lapse imaging revealed that knockdown of miR (zeige MYLIP Proteine)-219 function accelerates the growth of primary cilia, revealing a possible mechanistic link between miR (zeige MYLIP Proteine)-219-mediated regulation of apical Par (zeige AFG3L2 Proteine) proteins and Shh signaling.
Shh is not essential for the early activation of has2 (zeige HAS2 Proteine), but supports proper chondrogenic differentiation.
Opposing Shh and Fgf signals initiate nasotemporal patterning of the zebrafish retina.
Hedgehog signaling has a role in dental papilla formation and tooth size during zebrafish odontogenesis
Data indicate that the transgenic lines report Hedgehog pathway state in individual cells and with high sensitivity.
We further demonstrate that the elevated Hedgehog signaling in Sox11 (zeige SOX11 Proteine)-deficient zebrafish was caused by a large increase in shha transcription; indeed, suppressing Shha expression rescued the ocular phenotypes of sox11 (zeige SOX11 Proteine) morphants.
Pax6 (zeige PAX6 Proteine) has an evolutionarily conserved function in establishing the temporospatial expression of Shh in the mid-diencephalic organizer in vertebrates.
This gene encodes a protein that is instrumental in patterning the early embryo. It has been implicated as the key inductive signal in patterning of the ventral neural tube, the anterior-posterior limb axis, and the ventral somites. Of three human proteins showing sequence and functional similarity to the sonic hedgehog protein of Drosophila, this protein is the most similar. The protein is made as a precursor that is autocatalytically cleaved\; the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the developing embryo. Defects in this protein or in its signalling pathway are a cause of holoprosencephaly (HPE), a disorder in which the developing forebrain fails to correctly separate into right and left hemispheres. HPE is manifested by facial deformities. It is also thought that mutations in this gene or in its signalling pathway may be responsible for VACTERL syndrome, which is characterized by vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial and renal dysplasia, cardiac anomalies, and limb abnormalities. Additionally, mutations in a long range enhancer located approximately 1 megabase upstream of this gene disrupt limb patterning and can result in preaxial polydactyly.
sonic hedgehog protein
, sonic hedgehog
, hemimelic extra toes
, short digits
, sonic hedgehog homolog
, sonic hedgehog protein A