Gli3, Polyclonal Antibody

ACLS; DNA binding protein; GCPS; Gli 3; GLI family zinc finger 3; GLI Kruppel family member GLI 3; GLI Kruppel family member GLI3 (Greig cephalopolysyndactyly syndrome); GLI Kruppel family member GLI3; GLI3; GLI3 C-terminally truncated form; GLI3 form of 190 kDa; GLI3 form of 83 kDa; GLI3 full length protein; GLI3-190; GLI3-83; GLI3_HUMAN; GLI3FL; Glioma associated oncogene family zinc finger 3; Oncogene GLI3; PAP A; PAPA 1; PAPA; PAPA1; PAPB; PHS; PPD IV; PPDIV; Transcriptional activator GLI3; Transcriptional repressor GLI3R; Zinc finger protein GLI 3; Zinc finger protein GLI3

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Phospho-Gli3 (Ser664) Antibody detects endogenous levels of Gli3 only when phosphorylated at Ser664.

Purified rabbit serum by affinity purification via sequential chromatography on phospho- and non-phospho-peptide affinity columns.

Liquid; Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

1mg/ml (lot specific)

Store at -20 degree C. Stable for 12 months from date of receipt.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Manufactured in a lab with traceable raw materials manufactured on site. Coordinated product portfolio of antibodies, pairs, conjugates, recombinant proteins, and immunoassay materials available, please inquire.

Small volumes of anti-Gli3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: 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'.

Post Translational Modifications: Phosphorylated on multiple sites by protein kinase A (PKA) and phosphorylation by PKA primes further phosphorylation by CK1 and GSK3. Phosphorylated by DYRK2 (in vitro). Phosphorylation is essential for its proteolytic processing. Transcriptional repressor GLI3R, a C-terminally truncated form, is generated from the full-length GLI3 protein (GLI3FL/GLI3-190) through proteolytic processing. This process requires PKA-primed phosphorylation of GLI3, ubiquitination of GLI3 and the presence of BTRC. GLI3FL is complexed with SUFU in the cytoplasm and is maintained in a neutral state. Without the Hh signal, the SUFU-GLI3 complex is recruited to cilia, leading to the efficient processing of GLI3FL into GLI3R. GLI3R formation leads to its dissociation from SUFU, allowing it to translocate into the nucleus, and repress Hh target genes. When Hh signaling is initiated, SUFU dissociates from GLI3FL and this has two consequences. First, GLI3R production is halted. Second, free GLI3FL translocates to the nucleus, where it is phosphorylated, destabilized, and converted to a transcriptional activator (GLI3A). Phosphorylated in vitro by ULK3.

Subunit Structure: The full-length GLI3 form (GLI3FL) interacts with SUFU and this interaction regulates the formation of either repressor or activator forms of GLI3. Its association with SUFU is regulated by Hh signaling and dissociation of the SUFU-GLI3 interaction requires the presence of the ciliary motor KIF3A (By similarity). Interacts with KIF7. The activator form of GLI3 (GLI3A) but not the repressor form (GLI3R) can interact with TRPS1. The phosphorylated form interacts with BTRC. Interacts with ZIC1. Interacts with ZIC3 (via C2H2-type domains 3, 4 and 5); the interaction enhances its transcriptional activity.

Similarity: Belongs to the GLI C2H2-type zinc-finger protein family.

Western Blot (WB), Immunohistochemistry (IHC), Peptide ELISA (EIA)

WB: 1:500-1:2000
IHC: 1:50-1:200
ELISA (Peptide): 1:20000-1:40000

At 1/100 staining mouse heart tissue sections by IHC-P. The tissue was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The tissue was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary

At 1/100 staining rat heart tissue sections by IHC-P. The tissue was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The tissue was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary

At 1/100 staining rat lung tissue sections by IHC-P. The tissue was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The tissue was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary

At 1/100 staining human kidney tissue sections by IHC-P. The tissue was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The tissue was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary

Western blot analysis of extracts of Human Lung tissue sample,using Phospho-Gli3 (Ser664) Antibody.

Observed: 170 kDa
Predicted: 170 kDa

GLI family zinc finger 3

transcriptional activator GLI3

Transcriptional activator GLI3

GLI3-190; GLI3FL; GLI3-83  [Similar Products]

GLI3_HUMAN

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

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