Anti-PAH抗体(ab106805)
Key features and details
- Goat polyclonal to PAH
- Suitable for: WB
- Reacts with: Rat, Human
- Isotype: IgG
概述
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产品名称
Anti-PAH抗体
参阅全部 PAH 一抗 -
描述
山羊多克隆抗体to PAH -
宿主
Goat -
经测试应用
适用于: WBmore details -
种属反应性
与反应: Rat, Human
预测可用于: 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). -
阳性对照
- WB: Human liver and rat kidney tissue lysates.
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常规说明
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
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA -
Concentration information loading...
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纯度
Immunogen affinity purified -
纯化说明
ab106805 is purified from Goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab106805于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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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. |
说明 |
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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. |
靶标
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通路
Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6. -
疾病相关
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. -
序列相似性
Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
Contains 1 ACT domain. - Information by UniProt
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数据库链接
- 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 -
别名
- PAH antibody
- PH antibody
- PH4H_HUMAN antibody
see all
图片
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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
数据表及文件
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SDS download
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Datasheet download
文献 (0)
ab106805 尚未被引用在任何文献中。