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Data suggest that negative feedback mediated by GLI3 (GLI (show GLI1 ELISA Kits)-Kruppel family member) acts to finely tune SHH (sonic hedgehog (show SHH ELISA Kits)) signaling. During medulloblastoma (MB) formation, nerve tissue cells appear to express nestin (show NES ELISA Kits) which hyperactivates SHH (show SHH ELISA Kits) signaling by abolishing negative feedback by GLI3. Restoration of intrinsic negative feedback by repressing nestin (show NES ELISA Kits) expression represents a promising approach to treat MB. [REVIEW]
the first report of the assessment of the frequency of GLI3/SHH (show SHH ELISA Kits)/preZRS/ZRS in Chinese polydactyly patients to show any higher possibility of mutations or variants for the 4 genes or sequences in China
Gli3 and Teashirt3 (show ZNF537 ELISA Kits) might play an important role in the normal development of the ureter.
a novel GLI3 mutation c.714T>A (p.Y238*) was identified in a Chinese family with pre-axial polydactyly. Our results broadened the phenotypic spectrum of GLI3 mutations and demonstrated the feasibility of WES in clinical application of molecular diagnosis.
we describe an ~5 kb deletion within the SHH (show SHH ELISA Kits) repressor GLI3 in two patients with bilateral tibial hemimelia. This deletion results in a truncated GLI3 protein that lacks a DNA-binding domain and cannot repress hedgehog (show SHH ELISA Kits) signaling.
2 independent cases of GLI3 morphopathies presented: one is a familial case of Greig Cephalopolysyndactyly Syndrome and the other a non-syndromic case of post-axial polydactyly, both are caused due to a truncation mutation at C-terminal of GLI3
Gene silencing of GLI3 using RNA inference stimulated the growth of human Sertoli cells. miR (show MLXIP ELISA Kits)-133b promoted the proliferation of human Sertoli cells by targeting GLI3.
We report on a patient with GCPS caused by a novel GLI3 mutation.
Identify mutations tha (show SUFUH ELISA Kits)t in (show GLI1 ELISA Kits)crease (show SOX10 ELISA Kits) GLI activity in patients with Hirschsprung disease.
To date, at least ten loci and four non-syndromic polydactyly-causing genes, including the GLI3 gene, the ZNF141 gene, the MIPOL1 (show MIPOL1 ELISA Kits) gene and the PITX1 (show PITX1 ELISA Kits) gene, have been identified. (Review)
Gli2 (show GLI2 ELISA Kits) and Gli3 are dephosphorylated and activated in cilia and that impaired Gli2 (show GLI2 ELISA Kits) and Gli3 processing in Ta3 (show HSP90B1 ELISA Kits) mutant is at least in part due to a decrease in Gli2 (show GLI2 ELISA Kits) and Gli3 phosphorylation.
These results suggest that Tctn1 (show TCTN1 ELISA Kits), Tctn2 (show TCTN2 ELISA Kits), and Tctn3 (show TCTN3 ELISA Kits) are functionally divergent with respect to their role in ciliogenesis and Hedgehog (show SHH ELISA Kits) signaling but conserved in neural tube patterning and Gli3 processing.
Results indicate that the transcription factor Gli3 (Gli3)-mutant fetal liver (FL) had increased sonic hedgehog (Shh (show SHH ELISA Kits)) signaling resulting in decreased B cell development.
Our findings show how Nestin (show NES ELISA Kits) drives hedgehog (show SHH ELISA Kits) pathway-driven cancers and the tumor-promoting effects of Nestin (show NES ELISA Kits) were mediated by binding to Gli3, a zinc finger transcription factor (show OSR1 ELISA Kits) that negatively regulates hedgehog (show SHH ELISA Kits) signaling.
Gli3 gene is a direct target for repression by Tgifs during the neural tube patterning.
Sufu (show SUFUH ELISA Kits) is upregulated in active Shh (show SHH ELISA Kits) responding tissues and accompanies Gli (show GLI1 ELISA Kits) activators translocating into and Gli (show GLI1 ELISA Kits) repressors out of the nucleus.
Gli2 (show GLI2 ELISA Kits)+/-;Gli3Delta699/+ mice can serve as a genetic mouse model for common DSD (show FADS1 ELISA Kits).
cAMP dependent protein kinase A (PKA) is a key intracellular factor mediating SHH (show SHH ELISA Kits) signaling through regulation of GLI3 processing.
Despite increased production of full-length GLI2 (show GLI2 ELISA Kits) and GLI3 isoforms, previously identified GLI (show GLI1 ELISA Kits) targets important for mandibular and glossal development (Foxf1 (show FOXF1 ELISA Kits), Foxf2 (show FOXF2 ELISA Kits), Foxd1 (show FOXD1 ELISA Kits) and Foxd2 (show FOXD2 ELISA Kits)) were transcriptionally downregulated in Kif3a (show KIF3A ELISA Kits)(f/f);Wnt1 (show WNT1 ELISA Kits)-Cre embryos.
T-box3 interacts with Kif7 (show KIF7 ELISA Kits) and is required for normal stoichiometry and function of a Kif7 (show KIF7 ELISA Kits)/Sufu (show SUFUH ELISA Kits) complex that regulates Gli3 stability and processing.
Zebrafish Gli3 functions as both an activator and a repressor in Hedgehog (show SHH ELISA Kits) signaling. In the eye, Gli3 is also required for proper ath5 (show ATOH7 ELISA Kits) expression and the differentiation of retinal ganglion cells.
The results provided evidence that polymorphisms in the GLI3 gene are associated with growth traits, and may be used for marker-assisted selection in beef cattle breeding program.
This gene encodes a protein which belongs to the C2H2-type zinc finger proteins subclass of the Gli family. They are characterized as DNA-binding transcription factors and are mediators of Sonic hedgehog (Shh) signaling. The protein encoded by this gene localizes in the cytoplasm and activates patched Drosophila homolog (PTCH) gene expression. It is also thought to play a role during embryogenesis. Mutations in this gene have been associated with several diseases, including Greig cephalopolysyndactyly syndrome, Pallister-Hall syndrome, preaxial polydactyly type IV, and postaxial polydactyly types A1 and B.
GLI-Kruppel family member GLI3
, glioma-associated oncogene family zinc finger 3
, oncogene GLI3
, transcriptional activator GLI3
, zinc finger protein GLI3
, GLI-Kruppel family member GLI3 (Greig cephalopolysyndactyly syndrome)
, GLI3 full length protein
, neural specific DNA binding protein
, neural-specific DNA-binding protein xGLI3
, GLI-Kruppel family member 3
, GLI3 form of 190 kDa
, anterior digit pattern deformity
, extra toes
, polydactyly Nagoya
, zinc finger transcription factor Gli3
, GLI family zinc finger 3