Loading...
You have changed your country from
to
. Please be aware that this will change the currency in the purchasing process.
Products:Epigenetics and Nuclear Signaling >> Transcription >> Domain Families >> Zinc Finger
Overview
Product name
Anti-Gli3 antibody
See all Gli3 products (5) ...
Description
Rabbit polyclonal to Gli3
Tested applications
ICC/IF, IHC-Frmore details
Cross reactivity
Reacts with
Mouse
Predicted to work with
Rat, Chicken, Human, Xenopus laevis
Immunogen
Synthetic peptide: NEDESPGQTYHRERRNA-C conjugated to KLH, corresponding to amino acids 41-57 of Human GLI3.
NEDESPGQTY HRERRNA-C
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage buffer
Preservative: 0.01% Sodium Azide
Constituents: 0.15M Sodium chloride, 0.02M Potassium phosphate. pH 7.2
Concentration
Concentration information loading...
Purity
Immunogen affinity purified
Clonality
Polyclonal
Isotype
IgG
Research areas
Stem Cells >> Signaling Pathways >> Hedgehog >> Nuclear
Neuroscience >> Neurology process >> Neural Signal Transduction
Epigenetics and Nuclear Signaling >> Transcription >> Domain Families >> Zinc Finger
Applications
Show applications key
Our Abpromise guarantee covers the use of ab6050 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
ICC/IF: Use a concentration of 1 µg/ml(See Abreview.)
IHC-Fr: 1/100
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).
Alternative names
- ACLS antibody
- DNA binding protein antibody
- GCPS antibody
see all
Database links
- Entrez Gene: 420769 Chicken
- Entrez Gene: 2737 Human
- Entrez Gene: 14634 Mouse
- Entrez Gene: 140588 Rat
- Omim: 165240 Human
- SwissProt: Q9IA31 Chicken
- SwissProt: P10071 Human
- SwissProt: Q61602 Mouse
see all
Anti-Gli3 antibody images:
Western blot - Gli3 antibody (ab6050)
Western blot for Gli3 antibody (ab6050) at 1/500 tested on the following tissue lysates:
Lane 1 : Human Brain
Lane 2: Human Lung
Lane 3: Human Spleen
Lane 4: Mouse Brain
Lane 5: Mouse Lung
Secondary ab: Goat anti-rabbit IgG ab6721 (1/5000)
Exposure time: 3 minutes
Expected molecular weight: 2 isoforms of Gli3 exist, one is the full length 170-190kDa and the other is a truncated isoform at ~80kDa.
Cell lysates were loaded at 20
NB: This image shows the truncated isoform of Gli3 is detected by ab6050 in human lung (we do not know the identity of the 120-130kDa band). It was not possible to detect Gli3 immunoreactivity by WB in the other lysates tested. This is likely due to low expression/abundance of Gli3 in these tissue lysates. Furthermore, Gli3 expression is likely to be developmentally regulated and induced, making it difficult to succesfully detect in whole tissue homogenates. The staining on the WB shows low background and minimal cross reactivity in whole homogenates. The 50kDa band is likely due to non-specific binding of secondary antibody.
Immunohistochemistry (Frozen sections) - Gli3 antibody (ab6050)
Gil3 expression in the radial glia of the developing mouse neocortex.
ab6050 Rabbit polyclonal to Gil3 on Frozen sections of mouse E12.5 whole brain, showing staing of the radial glia, of the developing neocortex. Sections were paraformaldehyde fixed, prior to heat mediated antigen retrieval in citric acid, and 14 hours incubation with ab6050 (1/100).
This image is courtesy of an Abreview submitted by Dr Dean Griffiths
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Gli3 antibody (ab6050)
ab6050 at 0.625 µg/ml staining human glioblastoma.
Immunocytochemistry/ Immunofluorescence-Gli3 antibody(ab6050)
ICC/IF image of ab6050 stained HepG2 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab6050, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
References for Anti-Gli3 antibody (ab6050)
This product has been referenced in:
- Sehgal Ret al. BMP7 and SHH regulate Pax2 in mouse retinal astrocytes by relieving TLX repression. Dev Biol 332:429-43 (2009). ICC/IF; Rat.Read more (PubMed: 19505455) »
- Sehgal Ret al. BMP7 and SHH regulate Pax2 in mouse retinal astrocytes by relieving TLX repression. Dev Biol 332:429-43 (2009). ICC/IF; Mouse.Read more (PubMed: 19505455) »
See all 2 publications for this product
Publishing research using ab6050? 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"