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Methylation at K436 and K595 respectively by Set7 (zeige SETD7 Proteine) increases the stability and DNA binding ability of Gli3, resulting in an enhancement of Shh (zeige SHH Proteine) signaling activation.
Data suggest that negative feedback mediated by GLI3 (GLI (zeige GLI1 Proteine)-Kruppel family member) acts to finely tune SHH (sonic hedgehog (zeige SHH Proteine)) signaling. During medulloblastoma (MB) formation, nerve tissue cells appear to express nestin (zeige NES Proteine) which hyperactivates SHH (zeige SHH Proteine) signaling by abolishing negative feedback by GLI3. Restoration of intrinsic negative feedback by repressing nestin (zeige NES Proteine) expression represents a promising approach to treat MB. [REVIEW]
the first report of the assessment of the frequency of GLI3/SHH (zeige SHH Proteine)/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 (zeige ZNF537 Proteine) 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 (zeige SHH Proteine) 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 (zeige SHH Proteine) 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 (zeige MLXIP Proteine)-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 (zeige SUFUH Proteine)t in (zeige GLI1 Proteine)crease (zeige SOX10 Proteine) GLI activity in patients with Hirschsprung disease.
Gli2 and Gli3 are dephosphorylated and activated in cilia and that impaired Gli2 and Gli3 processing in Ta3 (zeige HSP90B1 Proteine) mutant is at least in part due to a decrease in Gli2 and Gli3 phosphorylation.
These results suggest that Tctn1 (zeige TCTN1 Proteine), Tctn2 (zeige TCTN2 Proteine), and Tctn3 (zeige TCTN3 Proteine) are functionally divergent with respect to their role in ciliogenesis and Hedgehog (zeige SHH Proteine) 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 (zeige SHH Proteine)) signaling resulting in decreased B cell development.
Our findings show how Nestin (zeige NES Proteine) drives hedgehog (zeige SHH Proteine) pathway-driven cancers and the tumor-promoting effects of Nestin (zeige NES Proteine) were mediated by binding to Gli3, a zinc finger transcription factor (zeige OSR1 Proteine) that negatively regulates hedgehog (zeige SHH Proteine) signaling.
Gli3 gene is a direct target for repression by Tgifs during the neural tube patterning.
Sufu (zeige SUFUH Proteine) is upregulated in active Shh (zeige SHH Proteine) responding tissues and accompanies Gli (zeige GLI1 Proteine) activators translocating into and Gli (zeige GLI1 Proteine) repressors out of the nucleus.
Gli2+/-;Gli3Delta699/+ mice can serve as a genetic mouse model for common DSD (zeige FADS1 Proteine).
cAMP dependent protein kinase A (PKA) is a key intracellular factor mediating SHH (zeige SHH Proteine) signaling through regulation of GLI3 processing.
Despite increased production of full-length GLI2 and GLI3 isoforms, previously identified GLI (zeige GLI1 Proteine) targets important for mandibular and glossal development (Foxf1 (zeige FOXF1 Proteine), Foxf2 (zeige FOXF2 Proteine), Foxd1 (zeige FOXD1 Proteine) and Foxd2 (zeige FOXD2 Proteine)) were transcriptionally downregulated in Kif3a (zeige KIF3A Proteine)(f/f);Wnt1 (zeige WNT1 Proteine)-Cre embryos.
T-box3 interacts with Kif7 and is required for normal stoichiometry and function of a Kif7/Sufu (zeige SUFUH Proteine) complex that regulates Gli3 stability and processing.
Zebrafish Gli3 functions as both an activator and a repressor in Hedgehog (zeige SHH Proteine) signaling. In the eye, Gli3 is also required for proper ath5 (zeige ATOH7 Proteine) 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