GLI3 Antibody
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中文名稱：GLI3兔多克隆抗體
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貨號：CSB-PA008881
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規格：￥1090
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：ACLS antibody; DNA binding protein antibody; GCPS antibody; Gli 3 antibody; GLI family zinc finger 3 antibody; GLI Kruppel family member GLI 3 antibody; GLI Kruppel family member GLI3 (Greig cephalopolysyndactyly syndrome) antibody; GLI Kruppel family member GLI3 antibody; GLI3 antibody; GLI3 C-terminally truncated form antibody; GLI3 form of 190 kDa antibody; GLI3 form of 83 kDa antibody; GLI3 full length protein antibody; GLI3-190 antibody; GLI3-83 antibody; GLI3_HUMAN antibody; GLI3FL antibody; Glioma associated oncogene family zinc finger 3 antibody; Oncogene GLI3 antibody; PAP A antibody; PAPA 1 antibody; PAPA antibody; PAPA1 antibody; PAPB antibody; PHS antibody; PPD IV antibody; PPDIV antibody; Transcriptional activator GLI3 antibody; Transcriptional repressor GLI3R antibody; Zinc finger protein GLI 3 antibody; Zinc finger protein GLI3 antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Synthesized peptide derived from the N-terminal region of Human GLI-3.
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
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產品提供形式：Liquid
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應用范圍：IHC, IF, ELISA
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推薦稀釋比：
Application Recommended Dilution IHC 1:100-1:300 IF 1:200-1:1000 ELISA 1:10000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Has a dual function as a transcriptional activator and a repressor of the sonic hedgehog (Shh) pathway, and plays a role in limb development. The full-length GLI3 form (GLI3FL) after phosphorylation and nuclear translocation, acts as an activator (GLI3A) while GLI3R, its C-terminally truncated form, acts as a repressor. A proper balance between the GLI3 activator and the repressor GLI3R, rather than the repressor gradient itself or the activator/repressor ratio gradient, specifies limb digit number and identity. In concert with TRPS1, plays a role in regulating the size of the zone of distal chondrocytes, in restricting the zone of PTHLH expression in distal cells and in activating chondrocyte proliferation. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'.
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基因功能參考文獻：
- We have determined two different mutations of GLI3 gene in two different cases, one of which is with GCPS and the other one is with PHS. A deletion mutation was detected in the proband with GCPS and his mother. Otherwise, we found that, unlike the previously reported, the mutation c.2437C>T, p.Q813X which was detected in the GLI3 gene caused typical PHS. PMID: 30226973
- Our preliminary results identified risk variants of GLI3 that are associated with NSCL/P susceptibility in a Chinese population. In particular, rs3801161 and its haplotypes rs3801161-rs7785287 displayed significant association with NSCL/P and survived Bonferroni correction for multiple comparisons. PMID: 28947718
- A novel GLI3 missense variant in a family that caused a spectrum of digital anomalies. All affected individuals that were tested harbored a c.1826G>A (p.(Cys609Tyr)) variant in GLI3. Functional studies of the murine p.Cys609Tyr GLI3 showed that the mutant protein is not efficiently processed to GLI3R, resulting in a full-length protein with basal transcriptional activity and submaximal pathway activation. PMID: 28884880
- The c.480dupC of the GLI3 gene probably underlies the synpolydactyly in this family. PMID: 28777844
- Methylation at K436 and K595 respectively by Set7 increases the stability and DNA binding ability of Gli3, resulting in an enhancement of Shh signaling activation. PMID: 27146893
- Data suggest that negative feedback mediated by GLI3 (GLI-Kruppel family member) acts to finely tune SHH (sonic hedgehog) signaling. During medulloblastoma (MB) formation, nerve tissue cells appear to express nestin which hyperactivates SHH signaling by abolishing negative feedback by GLI3. Restoration of intrinsic negative feedback by repressing nestin expression represents a promising approach to treat MB. [REVIEW] PMID: 28389227
- the first report of the assessment of the frequency of GLI3/SHH/preZRS/ZRS in Chinese polydactyly patients to show any higher possibility of mutations or variants for the 4 genes or sequences in China PMID: 28127823
- Gli3 and Teashirt3 might play an important role in the normal development of the ureter. PMID: 27279789
- 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. PMID: 27305983
- we describe an ~5 kb deletion within the SHH 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 signaling. PMID: 26791356
- 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 PMID: 26508445
- Gene silencing of GLI3 using RNA inference stimulated the growth of human Sertoli cells. miR-133b promoted the proliferation of human Sertoli cells by targeting GLI3. PMID: 26755652
- We report on a patient with GCPS caused by a novel GLI3 mutation. PMID: 25714367
- To date, at least ten loci and four non-syndromic polydactyly-causing genes, including the GLI3 gene, the ZNF141 gene, the MIPOL1 gene and the PITX1 gene, have been identified. (Review) PMID: 26515020
- from ESCs and induced pluripotent stem cells. SIGNIFICANCE STATEMENT: Our study presents a rapid and efficient protocol to generate human motoneurons from embryonic and induced pluripotent stem cells. PMID: 26290227
- This newly identified miR-506/Gli3 axis provides further insight into the pathogenesis of cervical cancer and indicates a potential novel therapeutic agent for the treatment of cervical cancer. PMID: 24608427
- Fu ubiquitination and cleavage is one of the key elements connecting the MID1-PP2A protein complex with GLI3 activity control PMID: 25278022
- the role of GLI3 in a significant fraction of patients with non-syndromic bilateral polydactyly affecting both hands and feet PMID: 24667698
- These data demonstrated an association between rs929387 of GLI3 and non-syndromic tooth agenesis in Chinese Han individuals. PMID: 24278334
- Data identifies a His601Arg mutation in the ZFD domain of GLI3 leading to phenotypic variability including an isolated limb phenotype in a jewish family. PMID: 25267529
- GLI3 mutation is associated with esophageal atresia. PMID: 24819706
- The findings of this study indicated that neuronal differentiation is associated with Gli3 expression in medulloblastoma cells, and that this feature predicts a favorable outcome for patients with medulloblastoma. PMID: 23889567
- No deletion or amplification of the entire GLI3 gene was found in a group of patients with isolated or syndrome-associated esophageal atresia. PMID: 23442119
- we have described the Gli3RD as an intrinsically disordered domain. PMID: 24146948
- it is proposed here that during vertebrate evolution the Gli3 expression control acquired multiple, independently acting, intronic enhancers for spatiotemporal patterning of CNS, limbs, craniofacial structures and internal organs PMID: 24102645
- This report supports the fact that the phenotypic variability exists in the GL13-associated disorders. There is a need to perform the GL13 mutational analysis in all cases of familial polysyndactyly, even without the typical triad of symptoms. PMID: 23334564
- High-expression of GLI3 was significantly associated with lymph node metastasis in lung adenocarcinoma. PMID: 23736020
- Partial gliosis in astrocytes is the result of an increase in transgenic GLI3 repressor activity. PMID: 24174682
- GLI3, fascin and TUBB3 are the most sensitive markers in Hodgkin lymphoma and anaplastic large cell lymphoma. PMID: 23454725
- Gli3-mediated hedgehog inhibition in human pluripotent stem cells initiates and augments developmental programming of adult hematopoiesis. PMID: 23293081
- activator Gli3 signal augments tumorigenicity of colorectal cancer irrespective of p53 status. PMID: 23176625
- The study data demonstrate an association between SNP rs929387 of the GLI3 gene and non-syndromic hypodontia in Chinese Han individuals. PMID: 22984994
- Intragenic GLI3 deletions may be responsible for Greig cephalopolysyndactyly syndrome and preaxial polydactyly type IV. PMID: 22903559
- document enhanced SHH pathway activation and GLI3-target gene induction coincident with impaired recruitment of CDK8 onto promoters of GLI3-target genes, but not non-GLI3-target genes PMID: 23091001
- AKT1-GLI3-VMP1 pathway mediates KRAS oncogene-induced autophagy in cancer cells. PMID: 22535956
- No expression of GLI3 gene was detected in the flexor hallucis longus of ICTEV patients or normal controls. PMID: 22678783
- a novel mutation of GLI3 causing various digital abnormalities PMID: 21320477
- loss of Gli3 signaling leads to disruption of the MDM2-p53 interaction and strongly potentiates p53-dependent cell growth inhibition in colon cancer cells PMID: 22227409
- GLI3 is strongly expressed by virtually 100% of the Hodgkin and Reed-Sternberg cells of Hodgkin lymphoma. PMID: 21531006
- association of intragenic GLI3 mutations with metopic synostosis PMID: 21326280
- Disruption of Gli3-Zic3 interaction in the critical period for ventral body wall formation may contribute to omphalocele phenotype in Cd chick model. PMID: 21069353
- Clinical and molecular findings of previously reported patients who had GLI3 mutations and genital abnormalities were reviewed. PMID: 21108399
- The phenotype spectrum of GL13 mutations is broader than encompassed by clinical diagnostic criteria in Greig cephalopolysyndactyly and Pallister-Hall syndromes (GCPS, PHS) patients than previously recognized. PMID: 20672375
- Pallister-Hall syndrome includes bifid epiglottis, hypothalamic hamartoblastoma, postaxial polydactyly, anal atresia, and occasionally laryngeal clefts. Mutations in the GLI3 gene can cause Pallister-Hall syndrome PMID: 20425471
- study reports on the novel association of trigonocephaly and polysyndactyly in two unrelated patients due to mutations within the last third (exon 14) and first third (exon 6) of the GLI3 gene, respectively PMID: 20583172
- These results not only demonstrate the high level of complexity in the genetic mechanisms controlling Gli3 expression, but also reveal the evolutionary significance of cis-acting regulatory networks of early developmental regulators in vertebrates. PMID: 20426846
- relationship between mutation points of GLI3 & Gli3 & resulting phenotypes in humans & mice described; discussed how reduced amounts of GLI3 protein or truncated mutant GLI3 protein disrupt development of limbs, head, face [review] PMID: 20201963
- Assays of deletion constructs revealed that the human-Fugu conserved sequences within the GLI3 intronic CNEs were essential but not sufficient for full-scale transcriptional activation PMID: 17426814
- GLI3 point mutations lead to misregulation of its subcellular localization PMID: 19829694
- The expression of Gli3, regulated by HOXD13, may play a role in idiopathic congenital talipes equinovarus. PMID: 19925654
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相關疾病：Greig cephalo-poly-syndactyly syndrome (GCPS); Pallister-Hall syndrome (PHS); Polydactyly, postaxial A1 (PAPA1); Polydactyly, postaxial B (PAPB); Polydactyly preaxial 4 (POP4)
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亞細胞定位：Nucleus. Cytoplasm. Cell projection, cilium. Note=GLI3FL is localized predominantly in the cytoplasm while GLI3R resides mainly in the nucleus. Ciliary accumulation requires the presence of KIF7 and SMO. Translocation to the nucleus is promoted by interaction with ZIC1.
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蛋白家族：GLI C2H2-type zinc-finger protein family
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組織特異性：Is expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium.
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數據庫鏈接：
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