概述

  • 产品名称
    重组人Gli3蛋白
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MEAQSHSSTTTEKKKVENSIVKCSTRTDVSEKAVASSTTSNEDESPGQTY HRERRNAITMQPQNVQGLSKVSEEPSTSSDERASLIKKEIHGSLPHVAEP SVPYRGTVFA
    • 分子量
      38 kDa including tags
    • 氨基酸
      1 to 110

技术指标

Our Abpromise guarantee covers the use of ab152429 in the following tested applications.

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

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 µg/µl.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • ACLS
    • DNA binding protein
    • GCPS
    • Gli 3
    • GLI family zinc finger 3
    • GLI Kruppel family member GLI 3
    • GLI Kruppel family member GLI3
    • GLI Kruppel family member GLI3 (Greig cephalopolysyndactyly syndrome)
    • GLI3
    • GLI3 C-terminally truncated form
    • GLI3 form of 190 kDa
    • GLI3 form of 83 kDa
    • GLI3 full length protein
    • GLI3-190
    • GLI3-83
    • GLI3_HUMAN
    • GLI3FL
    • Glioma associated oncogene family zinc finger 3
    • Oncogene GLI3
    • PAP A
    • PAPA
    • PAPA 1
    • PAPA1
    • PAPB
    • PHS
    • PPD IV
    • PPDIV
    • Transcriptional activator GLI3
    • Transcriptional repressor GLI3R
    • Zinc finger protein GLI 3
    • Zinc finger protein GLI3
    see all
  • 功能
    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'.
  • 组织特异性
    Is expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the GLI C2H2-type zinc-finger protein family.
    Contains 5 C2H2-type zinc fingers.
  • 翻译后修饰
    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.
  • 细胞定位
    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

图片

  • 12.5% SDS-PAGE analysis of ab152429 stained with Coomassie Blue.

文献

ab152429 has not yet been referenced specifically in any publications.

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