MABN754

Anti-PAH Antibody, clone 6H10.1

clone 6H10.1, from mouse

• 別名: Phenylalanine-4-hydroxylase, PAH, Phe-4-monooxygenase
• UNSPSCコード: 12352203
• eCl@ss: 32160702
• NACRES: NA.41
• クローン: 6H10.1, monoclonal
• application: IHC; WB
• 化学種の反応性: human
• テクニック: immunohistochemistry: suitable; western blot: suitable

特性

• 由来生物: mouse
• 品質水準: 100
• 抗体製品の状態: purified immunoglobulin
• 抗体製品タイプ: primary antibodies
• クローン: 6H10.1, monoclonal
• 化学種の反応性: human
• テクニック: immunohistochemistry: suitable; western blot: suitable
• アイソタイプ: IgG1κ
• NCBIアクセッション番号: NP_000268
• UniProtアクセッション番号: P00439
• 輸送温度: wet ice
• ターゲットの翻訳後修飾: unmodified
• 遺伝子情報: human ... PAH(5053)

詳細

詳細

PAH, also known as Phenylalanine-4-hydroxylase , Phe-4-monooxygenase, and encoded by the gene name PAH, belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Phenylalanine hydroxylase is the rate-limiting enzyme of the metabolic pathway that degrades excess phenylalanine. Phenylalanine hydroxylase (PheOH, alternatively PheH or PAH) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PheOH is one of three members of the pterin-dependent amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. During the reaction, molecular oxygen is heterolytically cleaved with sequential incorporation of one oxygen atom into BH4 and phenylalanine substrate. PAH has been associated with Phenylketonuria PKU, an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. Additioanlly, PAH has been associated with Non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA), a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Finally, PAH may play a role in the Hyperphenylalaninemia (HPA), a mildest form of phenylalanine hydroxylase deficiency. PAH is broadly expressed, with greatest levels in skeletal muscle followed by heart, brain, pancreas and testis.

免疫原

GST-tagged recombinant protein corresponding to human PAH.

アプリケーション

This Anti-PAH antibody is validated for use in WB, IH for the detection of PAH.

Western Blotting Analysis: 1.0 µg/mL from a representative lot detected PAH in 10 µg of human liver tissue lysate.
Immunohistochemistry Analysis: A 1:50-250 dilution from a representative lot detected PAH in human cerebral cortex and human liver tissue.

品質

Evaluated by Western Blotting in HepG2 cell lysate.

Western Blotting Analysis: 1.0 µg/mL of this antibody detected PAH in 10 µg of HepG2 cell lysate.

ターゲットの説明

~52 kDa observed

物理的形状

Format: Purified

その他情報

Concentration: Please refer to lot specific datasheet.

安全性情報

• 保管分類コード: 12 - Non Combustible Liquids
• WGK: WGK 1
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable