Anti-PAH 抗体 (ab88740)
Key features and details
- Mouse polyclonal to PAH
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
製品の概要
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製品名
Anti-PAH antibody
PAH 一次抗体 製品一覧 -
製品の詳細
Mouse polyclonal to PAH -
由来種
Mouse -
アプリケーション
適用あり: WB, IHC-Pmore details -
種交差性
交差種: Human -
免疫原
Recombinant full length protein within Human PAH. The exact immunogen sequence used to generate this antibody is proprietary information. If additional detail on the immunogen is needed to determine the suitability of the antibody for your needs, please contact our Scientific Support team to discuss your requirements.
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ポジティブ・コントロール
- WB: Human liver tissue lysate; PAH transfected 293T cell lysate IHC-P: Human kidney tissue
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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
pH: 7.40
Constituent: 100% PBS -
Concentration information loading...
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精製度
Protein A purified -
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
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Recombinant Protein
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ab88740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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WB | ||
IHC-P |
特記事項 |
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WB: Use at a concentration of 1 µg/ml. Detects a band of approximately 50 kDa (predicted molecular weight: 52 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
ターゲット情報
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パスウェイ
Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6. -
関連疾患
Defects in PAH are the cause of phenylketonuria (PKU) [MIM:261600]. PKU is an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol (normal concentration 100 mumol) which usually causes mental retardation (unless low phenylalanine diet is introduced early in life). They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.
Defects in PAH are the cause of non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA) [MIM:261600]. Non-PKU HPA is a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Non-PKU HPA is usually caused by the combined effect of a mild hyperphenylalaninemia mutation and a severe one.
Defects in PAH are the cause of hyperphenylalaninemia (HPA) [MIM:261600]. HPA is the mildest form of phenylalanine hydroxylase deficiency. -
配列類似性
Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
Contains 1 ACT domain. - Information by UniProt
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参照データベース
- Entrez Gene: 5053 Human
- Omim: 612349 Human
- SwissProt: P00439 Human
- Unigene: 603740 Human
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別名
- PAH antibody
- PH antibody
- PH4H_HUMAN antibody
see all
画像
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Anti-PAH antibody (ab88740) at 1 µg/ml + Human liver tissue lysate at 50 µg
Predicted band size: 52 kDa
Observed band size: 50 kDa why is the actual band size different from the predicted?
Additional bands at: <15 kDa. We are unsure as to the identity of these extra bands. -
All lanes : Anti-PAH antibody (ab88740) at 1 µg/ml
Lane 1 : PAH transfected 293T cell lysate
Lane 2 : Non transfected 293T cell lysate
Lysates/proteins at 25 µg per lane.
Predicted band size: 52 kDa
Observed band size: 50 kDa why is the actual band size different from the predicted? -
ab88740, at 3 µg/ml, staining PAH in formalin fixed, paraffin embedded Human kidney tissue by Immunoperoxidase/ Immunohistochemistry.
プロトコール
データシートおよび資料
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Datasheet download
参考文献 (0)
ab88740 は論文での使用が確認できていません。