Anti-Gli3 抗体 (ab6050)
Key features and details
- Rabbit polyclonal to Gli3
- Suitable for: ICC/IF, IHC-Fr, WB, IHC-P
- Reacts with: Mouse, Human
- Isotype: IgG
製品の概要
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製品名
Anti-Gli3 antibody
Gli3 一次抗体 製品一覧 -
製品の詳細
Rabbit polyclonal to Gli3 -
由来種
Rabbit -
特異性
A BLAST analysis was used to suggest cross reactivity with Gli-3 from human, chimpanzee, squirrel monkey, Xenopus laevis, chicken, dog and quail based on 100% sequence homology with the immunogen. Reactivity is also expected against homologues from mouse (94%) and rat (88%) based on partial homology. Reactivity with Gli-3 from other sources is not known.
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アプリケーション
適用あり: ICC/IF, IHC-Fr, WB, IHC-Pmore details -
種交差性
交差種: Mouse, Human
交差が予測される動物種: Rat, Chicken, Xenopus laevis -
免疫原
Synthetic peptide:
NEDESPGQTYHRERRNA-C
conjugated to KLH, corresponding to amino acids 41-57 of Human GLI3. -
特記事項
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
バッファー
Preservative: 0.01% Sodium azide
Constituents: 0.424% Tripotassium orthophosphate, 0.88% Sodium chloride -
Concentration information loading...
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精製度
Immunogen affinity purified -
特記事項(精製)
This affinity-purified antibody is directed against human Gli-3 protein. The product was affinity purified from monospecific antiserum by immunoaffinity purification. -
ポリ/モノ
ポリクローナル -
アイソタイプ
IgG -
研究分野
関連製品
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Compatible Secondaries
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Isotype control
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab6050の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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ICC/IF | (1) |
Use a concentration of 1 µg/ml. See Abreview.
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IHC-Fr | (1) |
Use at an assay dependent concentration.
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WB |
Use at an assay dependent concentration. Predicted molecular weight: 170 kDa. PubMed: 23293081
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IHC-P |
Use a concentration of 0.5 - 5 µg/ml.
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特記事項 |
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ICC/IF
Use a concentration of 1 µg/ml. See Abreview. |
IHC-Fr
Use at an assay dependent concentration. |
WB
Use at an assay dependent concentration. Predicted molecular weight: 170 kDa. PubMed: 23293081 |
IHC-P
Use a concentration of 0.5 - 5 µg/ml. |
ターゲット情報
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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
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参照データベース
- 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 -
別名
- ACLS antibody
- DNA binding protein antibody
- GCPS antibody
see all
画像
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ab6050 at 0.625 µg/ml staining human glioblastoma.
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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.
プロトコール
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (23)
ab6050 は 23 報の論文で使用されています。
- Shi H et al. Mutations in OSBPL2 cause hearing loss associated with primary cilia defects via sonic hedgehog signaling. JCI Insight 7:N/A (2022). PubMed: 35041619
- Shen M et al. GLI3 Promotes Invasion and Predicts Poor Prognosis in Colorectal Cancer. Biomed Res Int 2021:8889986 (2021). PubMed: 33506047
- Pan Y et al. Dysfunction of Shh signaling activates autophagy to inhibit trophoblast motility in recurrent miscarriage. Exp Mol Med 53:52-66 (2021). PubMed: 33390589
- Li L et al. Gli3 is a novel downstream target of miR-200a with an anti-?brotic role for progression of liver fibrosis in vivo and in vitro. Mol Med Rep 21:1861-1871 (2020). PubMed: 32319630
- He Y et al. CircZNF609 enhances hepatocellular carcinoma cell proliferation, metastasis, and stemness by activating the Hedgehog pathway through the regulation of miR-15a-5p/15b-5p and GLI2 expressions. Cell Death Dis 11:358 (2020). PubMed: 32398664