Anti-PAH抗体(ab88740)
Key features and details
- Mouse polyclonal to PAH
- Suitable for: WB, IHC-P
- Reacts with: 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
小鼠多克隆抗体to PAH -
宿主
Mouse -
经测试应用
适用于: WB, IHC-Pmore details -
种属反应性
与反应: 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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阳性对照
- WB: Human liver tissue lysate; PAH transfected 293T cell lysate IHC-P: Human kidney tissue
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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 or -80°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.40
Constituent: 100% PBS -
Concentration information loading...
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纯度
Protein A purified -
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ab88740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 | ||
IHC-P |
说明 |
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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.
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: 5053 Human
- Omim: 612349 Human
- SwissProt: P00439 Human
- Unigene: 603740 Human
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别名
- PAH antibody
- PH antibody
- PH4H_HUMAN antibody
see all
图片
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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.
数据表及文件
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Datasheet download
文献 (0)
ab88740 尚未被引用在任何文献中。