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由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
製品種目
Prestige Antibodies® Powered by Atlas Antibodies
フォーム
buffered aqueous glycerol solution
化学種の反応性
human
テクニック
immunohistochemistry: 1:50- 1:200
免疫原配列
GQQMLGQISATSHINIYQGPESCLPGAHGMGSQPSSLAVVRGYQPCASFGGSRRQAMPRDSLALQSGQLSDTSQTCRVNGIKMEMKGQPHPLCSNLQNYSGQFYDQTVGFSQQDTKAGSFSISDASCLLQGTSAKNSELLSPGA
UniProtアクセッション番号
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... GLI3(2737)
詳細
GLI family zinc finger 3 (GLI3) belongs to Gli family of DNA binding proteins. There are three Gli proteins (GLI1, GLI2 and GLI3), which contain a DNA-binding domain made up of five zinc fingers. Fingers 4 and 5 recognize the Gli-binding DNA sequence, and fingers 3, 4 and 5 interact closely with DNA. Their C-terminus consists of transcriptional activator domain (TAD), and in GLI3, the repressor domain (RD) is present at the N-terminus. In humans, GLI3 gene is present on chromosome 7p13, and spans 240kb. The encoded protein consists of 1596 amino acids. This protein is expressed during early stages of development.
免疫原
ジンクフィンガ-タンパク質GLI3のPrEST (protein epitope signature tag)抗原リコンビナントタンパク質。
アプリケーション
Prestige抗体®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。
生物化学的／生理学的作用
GLI family zinc finger 3 (GLI3) is involved in the sonic hedgehog (SHH) signaling in a downstream manner. It binds to the promoter region of genes downstream to it, and regulates their expression. In forebrain, this protein suppresses ventral cell types and regulates the specification of dorsal cell types. It suppresses digit formation when expressed in the anterior portion of the limb buds. Mutations in GLI3 gene usually lead to loss of function, and are a cause of multiple disorders, such as Greig Cephalopolysyndactyly Syndrome (GCPS), Acrocallosal Syndrome, Pallister-Hall Syndrome, Pre-axial Polydactyly type IV and Post-axial Polydactyly type A. Mutation in the N-terminal of GLI3 gene is linked to esophageal atresia. GLI3 is expressed in mesenchymal and epithelial layers during tooth development. SNP rs929387 of GLI3 is associated with tooth agenesis in Chinese Han population.
特徴および利点
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
関連事項
Corresponding Antigen APREST77454
物理的形状
PBS溶液 (pH 7.2, 40%グリセロ-ルおよび0.02%アジ化ナトリウム含有)。
法的情報
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責事項
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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保管分類コード
10 - Combustible liquids
WGK
WGK 1
引火点(°F)
Not applicable
引火点(℃)
Not applicable
個人用保護具 (PPE)
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
Jan Code
HPA005534-100UL:
HPA005534-25UL:
Haochen Liu et al.
PloS one, 8(11), e80860-e80860 (2013-11-28)
Tooth agenesis is one of the most common anomalies of human dentition. Recent studies suggest that a number of genes are related to both syndromic and non-syndromic forms of hypodontia. In a previous study, we observed that polymorphism in rs929387
Michael Volodarsky et al.
BMC medical genetics, 15, 110-110 (2014-10-01)
Polydactyly is a highly common congenital limb defect. Extra digits may appear as an isolated anomaly or as a part of a syndrome. Mutations in GLI3 have been shown to cause Greig cephalopolysyndactyly, Pallister-Hall syndrome and non-syndromic polydactyly. Genotype-phenotype correlation
Robert Tsanev et al.
PloS one, 8(10), e76972-e76972 (2013-10-23)
The transcription factor Gli3 is acting mainly as a transcriptional repressor in the Sonic hedgehog signal transduction pathway. Gli3 contains a repressor domain in its N-terminus from residue G106 to E236. In this study we have characterized the intracellular structure
B Wang et al.
Cell, 100(4), 423-434 (2000-02-29)
Ci/Gli zinc finger proteins mediate the transcriptional effects of Hedgehog protein signals. In Drosophila, Ci action as transcriptional repressor or activator is contingent upon Hedgehog-regulated, PKA-dependent proteolytic processing. We demonstrate that PKA-dependent processing of vertebrate Gli3 in developing limb similarly
A Wild et al.
Human molecular genetics, 6(11), 1979-1984 (1997-09-25)
Greig cephalopolysyndactyly syndrome (GCPS, MIM 175700) is a rare autosomal dominant developmental disorder characterized by craniofacial abnormalities and post-axial and pre-axial polydactyly as well as syndactyly of hands and feet. Human GLI3, located on chromosome 7p13, is a candidate gene
ライフサイエンス、有機合成、材料科学、クロマトグラフィー、分析など、あらゆる分野の研究に経験のあるメンバーがおります。.
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