Anti-Gli3 Antibody (CAB3300)
- SKU:
- CAB3300
- Product type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- ACLS
- GCPS
- GLI3-190
- GLI3FL
- PAP-A
- PAPA
- PAPA1
- PAPB
- PHS
- PPDIV
Description
| Product Name: | Gli3 Rabbit mAb |
| Product Code: | CAB3300 |
| Size: | 20uL, 50uL, 100uL |
| Synonyms: | ACLS, GCPS, GLI3-190, GLI3FL, PAP-A, PAPA, PAPA1, PAPB, PHS, PPDIV |
| Applications: | WB, IF |
| Reactivity: | Human, Rat |
| Host Species: | Rabbit |
| Immunogen: | A synthesized peptide derived from human Gli3 |
| Applications: | WB, IF |
| Recommended Dilutions: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| Reactivity: | Human, Rat |
| Positive Samples: | HeLa |
| Immunogen: | A synthesized peptide derived from human Gli3 |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 2737 |
| Uniprot: | P10071 |
| Calculated MW: | 190kDa |
| Observed MW: | 190KDa |
| UniProt Protein Function: | GLI3: 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'. Defects in GLI3 are the cause of Greig cephalo-poly- syndactyly syndrome (GCPS). GCPS is an autosomal dominant disorder affecting limb and craniofacial development. It is characterized by pre- and postaxial polydactyly, syndactyly of fingers and toes, macrocephaly and hypertelorism. Defects in GLI3 are a cause of Pallister-Hall syndrome (PHS). PHS is characterized by a wide range of clinical manifestations. It mainly associates central or postaxial polydactyly, syndactyly, and hypothalamic hamartoma. Malformations are frequent in the viscera, e.g. anal atresia, bifid uvula, congenital heart malformations, pulmonary or renal dysplasia. It is an autosomal dominant disorder. Defects in GLI3 are a cause of polydactyly postaxial type A1 (PAPA1). A trait characterized by an extra digit in the ulnar and/or fibular side of the upper and/or lower extremities. The extra digit is well formed and articulates with the fifth, or extra, metacarpal/metatarsal, and thus it is usually functional. Defects in GLI3 are a cause of polydactyly postaxial type B polydactyly (PAPB). A trait characterized by an extra digit in the ulnar and/or fibular side of the upper and/or lower extremities. The extra digit is not well formed and is frequently in the form of a skin. Defects in GLI3 are a cause of polydactyly preaxial type 4 (POP4). Polydactyly preaxial type 4 (i.e. polydactyly on the radial/tibial side of the hand/foot) covers a heterogeneous group of entities. In preaxial polydactyly type IV, the thumb shows only the mildest degree of duplication, and syndactyly of various degrees affects fingers 3 and 4. Belongs to the GLI C2H2-type zinc-finger protein family. |
| UniProt Protein Details: | Protein type:Transcription factor; C2H2-type zinc finger protein Chromosomal Location of Human Ortholog: 7p13 Cellular Component: nucleoplasm; transcriptional repressor complex; cytoplasm; nuclear speck; Srb-mediator complex; nucleus; cytosol; cilium Molecular Function:histone acetyltransferase binding; protein binding; histone deacetylase binding; metal ion binding; beta-catenin binding; chromatin binding; transcription factor activity Biological Process: developmental growth; radial glial cell differentiation in the forebrain; positive regulation of transcription, DNA-dependent; heart development; T cell differentiation in the thymus; anterior/posterior pattern formation; embryonic digestive tract morphogenesis; oligodendrocyte differentiation; lateral ganglionic eminence cell proliferation; embryonic gut development; positive regulation of neuroblast proliferation; transcription, DNA-dependent; optic nerve morphogenesis; negative thymic T cell selection; negative regulation of neuron differentiation; camera-type eye morphogenesis; positive regulation of chondrocyte differentiation; response to estrogen stimulus; positive regulation of transcription from RNA polymerase II promoter; embryonic digit morphogenesis; negative regulation of transcription, DNA-dependent; metanephros development; smoothened signaling pathway involved in spinal cord motor neuron cell fate specification; negative regulation of apoptosis; tongue development; axon guidance; wound healing; forebrain dorsal/ventral pattern formation; palate development; negative regulation of transcription from RNA polymerase II promoter; nose morphogenesis; negative regulation of cell proliferation; melanocyte differentiation; smoothened signaling pathway involved in ventral spinal cord interneuron specification; proximal/distal pattern formation; smoothened signaling pathway; in utero embryonic development; hippocampus development; limb morphogenesis; odontogenesis of dentine-containing teeth; embryonic skeletal morphogenesis; positive regulation of osteoblast differentiation; positive regulation of protein import into nucleus; cerebral cortex radial glia guided migration; ureteric bud branching; negative regulation of smoothened signaling pathway; protein processing; hindgut morphogenesis; positive regulation of alpha-beta T cell differentiation; negative regulation of alpha-beta T cell differentiation; lung development Disease: Polydactyly, Postaxial, Type A1; Hypothalamic Hamartomas; Pallister-hall Syndrome; Polydactyly, Preaxial Iv; Tracheoesophageal Fistula With Or Without Esophageal Atresia; Greig Cephalopolysyndactyly Syndrome |
| NCBI Summary: | 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. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P10071 |
| NCBI GenInfo Identifier: | 269849770 |
| NCBI Gene ID: | 2737 |
| NCBI Accession: | P10071.6 |
| UniProt Secondary Accession: | P10071,O75219, Q17RW4, Q75MT0, Q75MU9, Q9UDT5, Q9UJ39 A4D1W1, |
| UniProt Related Accession: | P10071 |
| Molecular Weight: | |
| NCBI Full Name: | Transcriptional activator GLI3 |
| NCBI Synonym Full Names: | GLI family zinc finger 3 |
| NCBI Official Symbol: | GLI3 |
| NCBI Official Synonym Symbols: | PHS; ACLS; GCPS; PAPA; PAPB; PAP-A; PAPA1; PPDIV; GLI3FL; GLI3-190 |
| NCBI Protein Information: | transcriptional activator GLI3; oncogene GLI3; zinc finger protein GLI3; GLI-Kruppel family member GLI3; glioma-associated oncogene family zinc finger 3 |
| UniProt Protein Name: | Transcriptional activator GLI3 |
| UniProt Synonym Protein Names: | GLI3 form of 190 kDa; GLI3-190; GLI3 full length protein; GLI3FL |
| Protein Family: | Zinc finger protein |
| UniProt Gene Name: | GLI3 |
| UniProt Entry Name: | GLI3_HUMAN |
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