Anti-Gli3 antibody (ab55437)

Overview

• Product nameAnti-Gli3 antibodySee all Gli3 primary antibodies ...
• Description
  Mouse monoclonal to Gli3
• Tested applicationsWB more details
• Species reactivity
  Reacts with: Recombinant Fragment
  Predicted to work with: Human
• Immunogen

  Recombinant fragment, corresponding to amino acids 1-111 of Human Gli3

Properties

• FormLiquid
• Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: None
  PBS, pH 7.2
• Concentration information loading...
• PurityProtein G purified
• Clonality Monoclonal
• IsotypeIgG2a
• Light chain typekappa
• Research Areas
  • Stem Cells
  • Signaling Pathways
  • Hedgehog
  • Nuclear

  • Epigenetics and Nuclear Signaling
  • Transcription
  • Domain Families
  • Zinc Finger

  • Neuroscience
  • Neurology process
  • Neural Signal Transduction

Applications

Target

• 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.
• Post-translational
  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.

Target information above from: UniProt accession P10071 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 2737 Human
  • Omim: 165240 Human
  • SwissProt: P10071 Human
  • Unigene: 21509 Human

• Alternative names
  ACLS antibodyDNA binding protein antibodyGCPS antibody

  Gli 3 antibodyGLI family zinc finger 3 antibodyGLI Kruppel family member GLI 3 antibodyGLI Kruppel family member GLI3 (Greig cephalopolysyndactyly syndrome) antibodyGLI Kruppel family member GLI3 antibodyGLI3 antibodyGLI3 C-terminally truncated form antibodyGLI3 form of 190 kDa antibodyGLI3 form of 83 kDa antibodyGLI3 full length protein antibodyGLI3-190 antibodyGLI3-83 antibodyGLI3_HUMAN antibodyGLI3FL antibodyGlioma associated oncogene family zinc finger 3 antibodyOncogene GLI3 antibodyPAP A antibodyPAPA 1 antibodyPAPA antibodyPAPA1 antibodyPAPB antibodyPHS antibodyPPD IV antibodyPPDIV antibodyTranscriptional activator GLI3 antibodyTranscriptional repressor GLI3R antibodyZinc finger protein GLI 3 antibodyZinc finger protein GLI3 antibody

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Anti-Gli3 antibody images

• 

  Western blot - Gli3 antibody (ab55437)
  Western blot against tagged recombinant protein immunogen using ab55437 Gli3 antibody at 1ug/ml. Predicted band size of immunogen is 38 kDa.

  This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.