Rabbit GLI3 Polyclonal Antibody | anti-GLI3 antibody

Rabbit Polyclonal to Human GLI3

Cat. Num. AAA240223

• Gene Names: GLI3; PHS; ACLS; GCPS; PAPA; PAPB; PAP-A; PAPA1; PPDIV; GLI3FL; GLI3-190
• Reactivity: Squirrel monkey, Chimpanzee, Chicken, Dog, Xenopus, Human, Quail
• Applications: Immunohistochemistry, Immunocytochemistry, Immunofluorescence, Western Blot, ELISA
• Purity: Immunoaffinity Purified
• Synonyms: GLI3; Polyclonal Antibody; Rabbit Polyclonal to Human GLI3; Anti-GLI3 Antibody (aa41-57) IHC-plus; ACLS; GCPS; GLI-Kruppel family member GLI3; GLI3 full length protein; GLI3-190; GLI family zinc finger 3; Gli-3; GLI3FL; PAP-A; PAPA1; PHS; PPDIV; Transcriptional activator GLI3; PAPA; PAPB; GLI3 form of 190 kDa; Oncogene GLI3; Human GLI3; anti-GLI3 antibody

• Host: Rabbit
• Reactivity: Squirrel monkey, Chimpanzee, Chicken, Dog, Xenopus, Human, Quail
• Clonality: Polyclonal
• Specificity: Amino acids 41-57 of human Gli-3 protein.
• Purity/Purification: Immunoaffinity Purified
• Form/Format: 0.02 M potassium phosphate, 0.15 M sodium chloride, pH 7.2, 0.1% sodium azide.
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 1580

• Applicable Applications for anti-GLI3 antibody: Immunohistochemistry (IHC - Paraffin), Immunocytochemistry (ICC), Immunofluorescence (IF), Western Blot (WB), ELISA (EIA)
• Application Notes: ELISA (1:6000 - 1:30000), ICC, IF, IHC-P (1.25 ug/ml), WB (1:500 - 1:2000); Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary antibo...
• Target Species: Human
• Immunogen Description: Synthetic peptide from human GLI3.
• Immunogen Type: Synthetic peptide
• Immunogen: GLI3 antibody was raised against synthetic peptide from human GLI3.
• Antigen Modification: aa41-57
• Preparation and Storage: Long term: -20 degree C; Short term: +4 degree C. Avoid repeat freeze-thaw cycles.

Immunohistochemistry (IHC)

(Anti-GLI3 antibody IHC of human brain-glioblastoma. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 1.25 ug/ml.)

Immunofluorescence (IF)

(Anti-Gli-3 Antibody - Immunofluorescence. Anti-Gli-3 Antibody Immunofluorescence image showing MCF-7 cell staining of Anti-alpha-Tubulin (MOUSE) Monoclonal Antibody - in green and staining of Anti-Gli-3 (RABBIT) Antibody - in red.)

Immunofluorescence (IF)

(Anti-Gli-3 Antibody - Immunofluorescence. Anti-Gli-3 Antibody Immunofluorescence image one showing MCF-7 cell staining of Anti-alpha-Tubulin (MOUSE) Monoclonal Antibody - in green. Immunofluorescence image two showing MCF-7 cell staining of Anti-Gli-3 (RABBIT) Antibody - in red. Immunofluorescence image three showing MCF-7 cell superimposed staining of Anti-alpha-Tubulin (MOUSE) Monoclonal Antibody - in green and staining of Anti-Gli-3 (RABBIT) Antibody - in red.)

Western Blot (WB)

(Anti-Gli-3 Antibody - Western Blot. Western blot of anti-Gli-3 antibody shows detection of multiple bands in human lung lysate believed to be Gli-3. Lanes contain 20 ug of whole cell lysates from 1 - human brain, 2 - human lung, 3 - human spleen, 4 - mouse brain and 5 - mouse lung. While no recognizable staining can be seen on mouse tissue, human lung shows what may be truncated Gli-3 (~80kD). This identity of the strong band at ~50 kD is unknown. After blocking, the membrane was probed with the primary antibody diluted to 1:500. For detection use HRP Gt-a-Rabbit IgG. Detection of Gli-3 by western blot may be enhanced if nuclear extracts are used instead of whole cell lysates as the expression/abundance of Gli-3 is likely to be low. Furthermore, Gli3 expression is likely to be developmentally regulated and induced, making it difficult to detect in whole tissue homogenates.)

Related Product Information for anti-GLI3 antibody

Gli-3 plays a role in limb and brain development and has been implicated in the transduction of SHH signal. Defects in Gli-3 are the cause of GREIG CEPHALOPOLYSYNDACTYLY SYNDROME (GCPS); an autosomal dominant disorder affecting limb and craniofacial development. It is characterized by pre-and post-axial polydactyly and syndactyly of fingers and toes, macrocephaly and hypertelorism.

NCBI and Uniprot Product Information

• NCBI GI #: 119393899
• NCBI GeneID: 2737
• NCBI Accession #: NP_000159.3
• NCBI GenBank Nucleotide #: NM_000168.5
• UniProt Accession #: P10071; O75219; Q17RW4; Q75MT0; Q75MU9; Q9UDT5; Q9UJ39; A4D1W1
• Molecular Weight: 169,863 Da
• NCBI Official Full Name: transcriptional activator GLI3
• NCBI Official 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
• Protein Family: Zinc finger protein
• UniProt Gene Name: GLI3
• UniProt Synonym Gene Names: GLI3-190; GLI3FL; GLI3-83
• UniProt Entry Name: GLI3_HUMAN

NCBI Description

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 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; metal ion binding; histone deacetylase binding; beta-catenin binding; chromatin binding; transcription factor activity

Biological Process: radial glial cell differentiation in the forebrain; developmental growth; positive regulation of transcription, DNA-dependent; heart development; T cell differentiation in the thymus; anterior/posterior pattern formation; embryonic digestive tract morphogenesis; oligodendrocyte differentiation; embryonic gut development; lateral ganglionic eminence cell proliferation; 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; axon guidance; tongue development; 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; embryonic skeletal morphogenesis; odontogenesis of dentine-containing teeth; positive regulation of osteoblast differentiation; positive regulation of protein import into nucleus; ureteric bud branching; cerebral cortex radial glia guided migration; negative regulation of smoothened signaling pathway; protein processing; hindgut morphogenesis; positive regulation of alpha-beta T cell differentiation; lung development; negative regulation of alpha-beta T cell differentiation

Disease: Polydactyly, Postaxial, Type A1; Hypothalamic Hamartomas; Polydactyly, Preaxial Iv; Pallister-hall Syndrome; Tracheoesophageal Fistula With Or Without Esophageal Atresia; Greig Cephalopolysyndactyly Syndrome