antibody

Anti-Gli3 antibody

MBS175299

0.1 mg

280 USD

polyclonal

rabbit

nonconjugated

human, rat

Antibody

Anti-Gli3 antibody

[Gli3]

[Transcriptional activator GLI3; GLI family zinc finger 3; 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]

[Transcriptional activator GLI3; Transcriptional activator GLI3; transcriptional activator GLI3; oncogene GLI3; zinc finger protein GLI3; GLI-Kruppel family member GLI3; glioma-associated oncogene family zinc finger 3; GLI family zinc finger 3; GLI3 form of 190 kDa; GLI3-190; GLI3 full length protein; GLI3FL]

[GLI3]

[GLI3; GLI3; PHS; ACLS; GCPS; PAPA; PAPB; PAP-A; PAPA1; PPDIV; GLI3FL; GLI3-190; GLI3-190; GLI3FL; GLI3-83]

GLI3_HUMAN

Polyclonal

IgG

Rabbit

Human, Rat. Predicted to work with: Mouse

1580

No cross reactivity with other proteins.

Immunogen affinity purified.

Lyophilized. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na 2 HPO 4 , 0.05mg Thimerosal, 0.05mg NaN 3 .

At -20°C for one year. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

Western Blotting:. Concentration: 0.1-0.5 ug/mL. Tested Species: Hu, Rat. Predicted Species: Ms. Tested Species: In-house tested species with positive results. Predicted Species: Species predicted to be fit for the product bases on sequence similarities. Other applications have not been tested. Optimal dilutions should be determined by end users.

Western Blot (WB)

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human GLI3 (40-57aa SNEDESPGQTYHRERRNA), different from the related rat sequence by two amino acids, and from the related mouse sequence by one amino acid.

Reconstitution: 0.2ml of distilled water will yield a concentration of 500ug/ml.

Description: Rabbit IgG polyclonal antibody for Transcriptional activator GLI3(GLI3) detection. Tested with WB in Human, Mouse, Rat. Background: GLI3(Gli-kruppel family member 3), also called ONCOGENE GLI3, encodes a member of the zinc finger gene family related to Kruppel, a gene that is known to regulate development in Drosophila. The GLI3 gene is expressed as an 8.5-kb mRNA in tissues such as testis, myometrium, placenta, and lung, and the protein product(relative molecular mass, 190,000) shows sequence-specific DNA binding. The GLI3 gene is mapped on 7p14.1. GLI3 is homologous to the Drosophila cubitus interruptus(ci) gene product(Ci), which regulates the Patched(pct), gooseberry(gsb), and decapentaplegic(dpp) genes. PKA-dependent processing of vertebrate GLI3 in developing limb generates a potent repressor in a manner antagonized by apparent long-range signaling from posteriorly localized Sonic hedgehog protein. The high relative abundance and potency of GLI3 repressor suggested specialization of GLI3 and its products for negative hedgehog pathway regulation.Coimmunoprecipitation and immunoblot studies showed that GLI3 protein is polyubiquitinated and that its processing depends on proteasome activity. The findings suggested that BTRC is required for GLI3 processing.

269849770

P10071.6

P10071

Basal Cell Carcinoma Pathway (83113); Basal Cell Carcinoma Pathway (525); Endochondral Ossification Pathway (198812); Hedgehog Signaling Pathway (198835); Hedgehog Signaling Events Mediated By Gli Proteins Pathway (137912); Hedgehog Signaling Pathway (83063); Hedgehog Signaling Pathway (474); Pathways In Cancer (83105); CAMP Signaling Pathway (1017634); CAMP Signaling Pathway (1019520)

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]

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

0.1 mg

280 USD