FITC标记的转录因子Gli3抗体上海沪震实业有限公司

性能参数

产品名称: FITC标记的转录因子Gli3抗体
英文名称: Anti-Gli3/FITC
抗体货号: HZ-11565R-FITC
产品规格: 100ul
级    别: 分析纯, , 分析纯,
产品产地: 中国/上海
品牌商标: HZbscience
价    格: 2980元
抗原: Rabbit
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: 
FITCconjugate:FITC
应用范围: ICC=1:50-200 IF=1:50-200
更新时间: 2018/6/13 16:14:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Rabbit Anti-Gli3/FITC Conjugated antibody

FITC标记的转录因子Gli3抗体

 

产品编号 bs-11565R-FITC
英文名称 Anti-Gli3/FITC
中文名称 FITC标记的转录因子Gli3抗体
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 (Greig cephalopolysyndactyly syndrome); GLI Kruppel family member GLI3; 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 1; PAPA; PAPA1; PAPB; PHS; PPD IV; PPDIV; Transcriptional activator GLI3; Transcriptional repressor GLI3R; Zinc finger protein GLI 3; Zinc finger protein GLI3.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  信号转导  干细胞  转录调节因子  锌指蛋白  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Cow, Horse, Rabbit, Sheep, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 170kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Gli3 (481-570aa)
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
It has long been known that the overexpression of either Wnt-1 or the GLI proteins results in cancer; however, the molecular basis for this transformation was poorly understood. The Wnt-1 and GLI proteins have now been placed in a signaling cascade downstream of the mammalian homologs of the Drosophila hedgehog and patched proteins. The Drosophila segment polarity gene hedgehog (hh) encodes a secreted protein that appears to function in embryonic and imaginal disc patterning. The ptc gene, also identified as a Drosophila segment polarity gene, encodes the transmembrane protein patched, the expression of which is precisely regulated during embryonic development. Hedgehog has been shown to enhance the expression of the Wnt family of proteins through a signaling cascade involving the GLI transcription factors, while patched functions as a repressor opposing the effects of hedgehog. Mutations in the ptc gene, which result in unregulated hedgehog signaling, have been correlated with the most common type of cancer, basal cell carcinoma, which affects 750,000 individuals annually in the United States alone.

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'.

Subunit:
The full-length GLI3 form (GLI3FL) interacts with SUFU and this interaction regulates the formation of either repressor or activator forms of GLI3. Its association with SUFU is regulated by Hh signaling and dissociation of the SUFU-GLI3 interaction requires the presence of the ciliary motor KIF3A (By similarity). Interacts with KIF7. The activator form of GLI3 (GLI3A) but not the repressor form (GLI3R) can interact with TRPS1. The phosphorylated form interacts with BTRC. Interacts with ZIC1. Interacts with ZIC3 (via C2H2-type domains 3, 4 and 5); the interaction enhances its transcriptional activity.

Subcellular Location:
Nucleus. Cytoplasm. Cell projection

Tissue Specificity:
Is expressed in a wide variety of normal adult tissues, including lung, colon, spleen, placenta, testis, and myometrium.

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.

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.

Similarity:
Belongs to the GLI C2H2-type zinc-finger protein family.
Contains 5 C2H2-type zinc fingers.

Database links:

Entrez Gene: 420769 Chicken

Entrez Gene: 463369 Chimpanzee

Entrez Gene: 483244 Dog

Entrez Gene: 2737 Human

Entrez Gene: 14634 Mouse

Entrez Gene: 140588 Rat

Omim: 165240 Human

SwissProt: Q9IA31 Chicken

SwissProt: Q5IS56 Chimpanzee

SwissProt: P10071 Human

SwissProt: Q61602 Mouse

SwissProt: Q91660 Xenopus laevis

Unigene: 21509 Human

Unigene: 5098 Mouse



Important Note:
   

长期以来,无论是Wnt-1或GLI蛋白的过度表达都会导致癌症,然而,这种转化的分子基础却知之甚少。Wnt-1和GLI蛋白现在已被放置在果蝇刺猬和补丁蛋白的哺乳动物同源物下游的信号级联。果蝇片段极性基因Hedgehog(HH)编码分泌蛋白,其在胚胎和想象的椎间盘构型中发挥作用。PTC基因,也被鉴定为果蝇片段极性基因,编码跨膜蛋白补丁,其表达在胚胎发育过程中被精确调节。刺猬已被证明通过WLNT蛋白家族的表达通过涉及GLI转录因子的信号级联来增强,而补丁功能作为抑制Hedgehog效应的阻遏物。PTC基因的突变,导致未调节的刺猬信号,已与最常见的类型的癌症,基底细胞癌,每年仅影响750000个人在美国。

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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