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Anti-Gli3 antibody (ab69838)

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    Overview

    Product name

    Anti-Gli3 antibody
    See all Gli3 products (5) ...

    Description

    Rabbit polyclonal to Gli3

    Tested applications

    WBmore details

    Cross reactivity

    Reacts with

    Mouse, Human

    Predicted to work with

    Rat, Chicken, Chimpanzee, Rhesus monkey

    Immunogen

    Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Gli3.

    (Peptide available as ab69917.)

    Positive control

    This antibody gave a positive signal in the following Lysates: Mouse Pancreas Tissue, Mouse Liver Tissue, MEF1 Whole Cell, TE 671 Whole Cell, RAW 264.7 Whole Cell

    Properties

    Form

    Liquid

    Storage instructions

    Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Storage buffer

    Preservative: 0.02% Sodium Azide
    Constituents: 1% BSA, PBS, pH 7.4

    Concentration

    Concentration information loading...

    Purity

    Immunogen affinity purified

    Clonality

    Polyclonal

    Isotype

    IgG

    • Western blot - Gli3 antibody (ab69838)Western blot - Gli3 antibody (ab69838) image (enlarge)

    Applications

    Show applications key

    Our Abpromise guarantee covers the use of ab69838 in the following tested applications.

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

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      WB

       WB: Use a concentration ...Read more →

      WB: Use a concentration of 1 µg/mlDetects a band of approximately 145 kDa (predicted molecular weight: 160 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.

    Target information above from: UniProt accessionP10071 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010).

    Information by UniProt

    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

    Anti-Gli3 antibody images:

      Western blot - Gli3 antibody (ab69838)

    Western blot - Gli3 antibody (ab69838)

    All lanes : Anti-Gli3 antibody (ab69838) at 1 µg/ml

    Lane 1 : Pancreas (Mouse) Tissue Lysate
    Lane 2 : Liver (Mouse) Tissue Lysate
    Lane 3 : MEF1 (Mouse embryonic fibroblast cell line) Whole Cell Lysate
    Lane 4 : TE 671 (Human Rhabdomyosarcoma) Whole Cell Lysate
    Lane 5 : RAW 264.7 (Mouse leukaemic monocyte macrophage cell line) Whole Cell Lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution

    Performed under reducing conditions.

    Predicted band size : 160 kDa
    Observed band size : 145 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 110 kDa,55 kDa,75 kDa. We are unsure as to the identity of these extra bands.

    References for Anti-Gli3 antibody (ab69838)

    ab69838 has not yet been referenced specifically in any publications.

    Publishing research using ab69838? Please let us know so that we can cite the reference in this datasheet

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