Anti-PAH 抗体 (ab106805)
Key features and details
- Goat polyclonal to PAH
- Suitable for: WB
- Reacts with: Rat, 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
Goat polyclonal to PAH -
由来種
Goat -
アプリケーション
適用あり: WBmore details -
種交差性
交差種: Rat, Human
交差が予測される動物種: Mouse, Chicken, Cow, Dog -
免疫原
Synthetic peptide:
ESRPSRLKKDE
by a Cysteine residue linker, corresponding to internal sequence amino acids 66-76 of Human PAH (NP_000268.1). -
ポジティブ・コントロール
- WB: Human liver and rat kidney tissue lysates.
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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. Avoid repeated freeze / thaw cycles. -
バッファー
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA -
Concentration information loading...
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精製度
Immunogen affinity purified -
特記事項(精製)
ab106805 is purified from Goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. -
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ab106805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 |
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 48 kDa (predicted molecular weight: 52 kDa).
1 hour primary incubation is recommended for this product. |
特記事項 |
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WB
Use a concentration of 0.1 - 0.3 µg/ml. Detects a band of approximately 48 kDa (predicted molecular weight: 52 kDa). 1 hour primary incubation is recommended for this product. |
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: 408024 Chicken
- Entrez Gene: 510583 Cow
- Entrez Gene: 475446 Dog
- Entrez Gene: 5053 Human
- Entrez Gene: 18478 Mouse
- Entrez Gene: 24616 Rat
- Omim: 612349 Human
- SwissProt: Q2KIH7 Cow
see all -
別名
- PAH antibody
- PH antibody
- PH4H_HUMAN antibody
see all
画像
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Anti-PAH antibody (ab106805) at 0.1 µg/ml + Human liver tissue lysate (35µg protein in RIPA buffer)
Developed using the ECL technique.
Predicted band size: 52 kDa -
Anti-PAH antibody (ab106805) at 0.3 µg/ml + Rat kidney tissue lysate (35µg protein in RIPA buffer)
Developed using the ECL technique.
Predicted band size: 52 kDa
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 (0)
ab106805 は論文での使用が確認できていません。