Testing Data
(Detection limit for recombinant GST tagged GLI3 is 3 ng/ml as a capture antibody.)
Mouse GLI3 Monoclonal Antibody | anti-GLI3 antibody
GLI3 (GLI-Kruppel Family Member GLI3, ACLS, GCPS, PAP-A, PAPA, PAPA1, PAPB, PHS, PPDIV) (AP)
Testing Data
(Detection limit for recombinant GST tagged GLI3 is 3 ng/ml as a capture antibody.)
Testing Data
(Detection limit for recombinant GST tagged GLI3 is 3 ng/ml as a capture antibody.)
NCBI and Uniprot Product Information
Uniprot Description
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.
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
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Product Notes
The GLI3 gli3 (Catalog #AAA6165199) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's GLI3 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the GLI3 gli3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GLI3, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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