Recombinant
RabMAb

Anti-Gli3 antibody [EPR4594] (ab181130)

Overview

  • Product name
    Anti-Gli3 antibody [EPR4594]
    See all Gli3 primary antibodies
  • Description
    Rabbit monoclonal [EPR4594] to Gli3
  • Tested applications
    Suitable for: ICC/IF, WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Chimpanzee
  • Immunogen

    Recombinant fragment within Human Gli3 aa 1300-1500. The exact sequence is proprietary.
    Database link: P10071

  • Positive control
    • 293 lysate, 293 cells
  • General notes

    This product is a recombinant rabbit monoclonal antibody.

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents

Properties

Associated products

Applications

Our Abpromise guarantee covers the use of ab181130 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF 1/100.
WB 1/1000 - 1/2000. Predicted molecular weight: 190 kDa.

Target

  • Function
    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'.
  • Tissue specificity
    Is expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium.
  • Involvement in disease
    Defects in GLI3 are the cause of Greig cephalo-poly-syndactyly syndrome (GCPS) [MIM:175700]. 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) [MIM:146510]. 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 type A1/B postaxial polydactyly (PAPA1/PAPB) [MIM:174200, 603596]. PAPA in humans is an autosomal dominant 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 preaxial type 4 (POP4) [MIM:174700]. 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.
    Defects in GLI3 are the cause of acrocallosal syndrome (ACS) [MIM:200990]; also abbreviated ACLS. ACS is characterized by postaxial polydactyly, hallux duplication, macrocephaly, and absence of the corpus callosum, usually with severe developmental delay.
  • Sequence similarities
    Belongs to the GLI C2H2-type zinc-finger protein family.
    Contains 5 C2H2-type zinc fingers.
  • Post-translational
    modifications
    Phosphorylated on multiple sites by protein kinase A (PKA) and phosphorylation by PKA primes further phosphorylation by CK1 and GSK3. 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.
  • Cellular localization
    Nucleus. Cytoplasm. Cell projection > cilium. GLI3FL is localized predominantly in the cytoplasm while GLI3R resides mainly in the nucleus. Ciliary accumulation requires the presence of KIF7 and SMO. Translocation to the nucleus is promoted by interaction with ZIC1.
  • Information by UniProt
  • Database links
  • Alternative names
    • 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
    see all

Images

  • Immunofluorescence analysis of 293 cells (fixative 4% paraformaldehyde) labeling Gli3 with ab181130 at a 1/100 dilution. Goat anti rabbit IgG (Alexa Fluor 488™) secondary used at a 1/200 diution.

  • Anti-Gli3 antibody [EPR4594] (ab181130) at 1/1000 dilution + 293 lysate at 20 µg

    Secondary
    Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution

    Predicted band size : 190 kDa

References

ab181130 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Application
Western blot
Sample
Human Cell lysate - whole cell (Human prostate cancer cell)
Gel Running Conditions
Reduced Denaturing (10%)
Loading amount
30 µg
Specification
Human prostate cancer cell
Blocking step
Milk as blocking agent for 2 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Abcam user community

Verified customer

Submitted Sep 17 2015

Application
Immunocytochemistry/ Immunofluorescence
Sample
Human Cell (Human prostate cancer cell)
Permeabilization
Yes - PBS with 0.025%Triton
Specification
Human prostate cancer cell
Blocking step
Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 25°C
Fixative
Methanol
Username

Abcam user community

Verified customer

Submitted Jul 27 2015

Application
Immunoprecipitation
Sample
Human Cell lysate - whole cell (Prostate cancer)
Total protein in input
2000 µg
Immuno-precipitation step
Protein G
Specification
Prostate cancer
Username

Abcam user community

Verified customer

Submitted Jul 22 2015

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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