Phenylalanine Hydroxylase 抗体
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Quick Overview for Phenylalanine Hydroxylase 抗体 (ABIN498690)
抗原
See all Phenylalanine Hydroxylase 抗体宿主
克隆类型
标记
应用范围
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特异性
- This antibody detects endogenous levels of PAH protein. (region surrounding Arg400)
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交叉反应 (详细)
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Species reactivity (expected):Mouse and Rat.
Species reactivity (tested):Human. -
纯化方法
- Affinity chromatography
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纯度
- > 95 % by SDS-PAGE
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应用备注
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Western Blot: 1/500 - 1/1000. Immunohistochemistry: 1/50 - 1/200.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
限制
- 仅限研究用
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浓度
- 1,0 mg/mL
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缓冲液
- Phosphate buffered saline (PBS), pH 7.2., 0.05 % sodium azide
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储存液
- Sodium azide
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注意事项
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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注意事项
- Avoid repeated freezing and thawing.
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储存条件
- 4 °C/-20 °C
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储存方法
- Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
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- Phenylalanine Hydroxylase
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别名
- PAH
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物质类
- Chemical
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背景
- The PAH gene encodes the enzyme phenylalanine hydroxylase (PAH), which converts phenylalanine to tyrosine and is the rate-limiting enzyme in phenylalanine catabolism. Mammalian PAH is a soluble, homotetrameric protein which is abundantly expressed in human liver. Deficiency of PAH activity results in the autosomal recessive disorder phenylketonuria (PKU), which is characterized by mental retardation unless a low phenylalanine diet is introduced early in life. The PAH gene, which maps to human chromosome 12q23.2, contains all the genetic information necessary to code for functional PAH, demonstrating that a single gene is involved in the classic disease phenotype. Numerous mutations can impair the PAH gene, which result in decreased enzyme activity and give rise to varying degrees of PKU. Multiple isozymes of PAH have been reported to exist, but these are most likely allelic variants of PAH that produce protein subunits with slightly different charge and electrophoretic migration.Synonyms: PH, PKU, PKU1, Phe-4-monooxygenase, Phenylalanine 4-monooxygenase, Phenylalanine Hydroxylase, Phenylalanine-4-hydroxylase
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分子量
- approx. 55 kDa
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基因ID
- 5053
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NCBI登录号
- NP_000268
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UniProt
- P00439
抗原
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