Key features and details
- Rabbit polyclonal to Gli3
- Suitable for: WB, IP
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Gli3 antibody
See all Gli3 primary antibodies
DescriptionRabbit polyclonal to Gli3
Tested applicationsSuitable for: WB, IPmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Rabbit, Horse, Guinea pig, Cow, Dog, Pig, Chimpanzee, Rhesus monkey, Gorilla, Orangutan, Platypus
Synthetic peptide, corresponding to a region within amino acids 875-925 of Human Gli3 using the numbering given in entry NP_000159.3.
- Whole cell lysate from 293T and HeLa
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferpH: 7
Preservative: 0.09% Sodium azide
Constituent: 99% Tris citrate/phosphate
pH 7 to 8
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab123495 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/2000 - 1/10000. Predicted molecular weight: 170 kDa.|
|IP||Use at 2-10 µg/mg of lysate.|
FunctionHas 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 specificityIs expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium.
Involvement in diseaseDefects 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 similaritiesBelongs to the GLI C2H2-type zinc-finger protein family.
Contains 5 C2H2-type zinc fingers.
modificationsPhosphorylated 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 localizationNucleus. 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
- ACLS antibody
- DNA binding protein antibody
- GCPS antibody
All lanes : Anti-Gli3 antibody (ab123495) at 0.1 µg/ml
Lane 1 : Whole cell lysate from 293T at 50 µg
Lane 2 : Whole cell lysate from 293T at 15 µg
Lane 3 : Whole cell lysate from Jurkat at 50 µg
Lane 4 : Whole cell lysate from HeLa at 50 µg
Developed using the ECL technique.
Predicted band size: 170 kDa
Exposure time: 30 seconds
Immunoprecipitation from 293T whole cell lysate (1 mg for IP, 20% of IP loaded), using ab123495 at 6 µg/mg lysate for IP
Subsequent Western blot detection used ab123495 at 1 µg/ml.
Detection: Chemiluminescence with an exposure time of 30 seconds.
ab123495 has been referenced in 1 publication.
- Collette NM et al. Sost and its paralog Sostdc1 coordinate digit number in a Gli3-dependent manner. Dev Biol 383:90-105 (2013). PubMed: 23994639