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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
形状
buffered aqueous glycerol solution
化学種の反応性
human
強化検証
independent
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
テクニック
immunohistochemistry: 1:500-1:1000
western blot: 0.04-0.4 μg/mL
免疫原配列
MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDVNLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPWFPRTIQELDRFANQILSYGAELDADHPG
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 ... PAH(5053)
詳細
Phenylalanine hydroxylase (PAH) gene spanning 90100 kb, with 13 exons, is mapped to human chromosome 12q23.2. The gene codes for non-heme iron enzyme, phenylalanine hydroxylase that belongs to the pteridine-dependent aromatic amino acid monooxygenase family.
免疫原
フェニルアラニンヒドロキシラーゼの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
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
生物化学的／生理学的作用
Phenylalanine hydroxylase (PAH) catalyzes the hydroxylation of phenylalanine to tyrosine in the presence of molecular oxygen and cofactor tetrahydrobiopterin (BH4). Mutation in the gene leads to the development of phenylketonuria (PKU) and hyperphenylalaninemia.
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 APREST83043
物理的形状
PBS溶液(pH 7.2, 40%グリセロールおよび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
試験成績書（COA）
製品のロット番号・バッチ番号を入力して、試験成績書（COA） を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
Prenatal diagnosis of Chinese families with phenylketonuria.
Liu N, et al.
Genetics and molecular research : GMR, 14, 14615-14628 (2015)
Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation
Andersen OA, et al.
Journal of Molecular Biology, 320(5), 1095-1108 (2002)
Normal values and age-dependent changes in GTP cyclohydrolase I activity in stimulated mononuclear blood cells measured by high-performance liquid chromatography
Hibiya M, et al.
Journal of Chromatography. B, Biomedical Sciences and Applications, 740(1), 35-42 (2000)
Robin A Williams et al.
The Clinical biochemist. Reviews, 29(1), 31-41 (2008-06-21)
Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resulting from deficiency of phenylalanine hydroxylase (PAH). Most forms of PKU and hyperphenylalaninaemia (HPA) are caused by mutations in the PAH gene on chromosome 12q23.2. Untreated PKU is
The catecholamine system in health and disease
Nagatsu T
Proceedings of the Japan Academy. Series B, Physical and Biological Sciences, 82(10), 388-415 (2006)
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