製品の概要

  • 製品名Anti-Gli3 antibody
    Gli3 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Gli3
  • アプリケーション適用あり: WB, ELISA, IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat, Chicken, Dog, Xenopus laevis, Chimpanzee, Monkey, Quail
  • 免疫原

    Synthetic peptide:

    NEDESPGQTYHRERRNA

    conjugated to KLH, corresponding to amino acids 41-57 of Human Gli3.

  • ポジティブ・コントロール
    • Human glioblastoma tissue

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab117099 in the following tested applications.

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

アプリケーション Abreviews 特記事項
WB 1/500 - 1/2000. Predicted molecular weight: 170 kDa.
ELISA 1/6000 - 1/30000.
IHC-P Use a concentration of 1.25 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

ターゲット情報

  • 機能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'.
  • 組織特異性Is expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium.
  • 関連疾患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.
  • 配列類似性Belongs to the GLI C2H2-type zinc-finger protein family.
    Contains 5 C2H2-type zinc fingers.
  • 翻訳後修飾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.
  • 細胞内局在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
  • 参照データベース
  • 別名
    • 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 画像

  • ab117099, at 1.25µg/ml, staining Gli3 in formalin-fixed, paraffin-embedded Human glioblastoma tissue by Immunohistochemistry. Following primary incubation the slide was incubated with biotinylated secondary antibody, alkaline phosphatase-streptavidin and chromogen.

Anti-Gli3 antibody (ab117099) 使用論文

ab117099 has not yet been referenced specifically in any publications.

Product Wall

Abreviews
Application Western blot
Loading amount 100 µg
Gel Running Conditions Reduced Denaturing (8% acrylamide gel)
Sample Rat Tissue lysate - whole (Liver)
Specification Liver
Treatment CCl4
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: RT°C
Username

Abcam user community

Verified customer

投稿 Jan 02 2014

Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Blocking step Dako, Protein Block as blocking agent for 30 minute(s) · Concentration: 100% · Temperature: RT°C
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Sodium Citrate, pH 6.0
Sample Rat Tissue sections (Liver)
Specification Liver
Permeabilization No
Fixative Formaldehyde
Username

Abcam user community

Verified customer

投稿 Jan 02 2014

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