Aminoacylase 1 抗体 (AA 1-408)
Quick Overview for Aminoacylase 1 抗体 (AA 1-408) (ABIN5775904)
抗原
See all Aminoacylase 1 (ACY1) 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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抗原表位
- AA 1-408
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免疫原
- Recombinant human ACY1 (1-408aa) purified from E. coli
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亚型
- IgG2b kappa
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 mg/mL
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储存条件
- 4 °C,-20 °C,-80 °C
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储存方法
- Can be stored at +2°C to +8°C for 1 week. For long term storage, aliquot and store at -20°C to -80°C. Avoid repeated freezing and thawing cycles.
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- Aminoacylase 1 (ACY1)
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别名
- Aminoacylase/ACY1
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背景
- Aminoacylase-1, also designated N-acyl-L-amino-acid amidohydrolase or ACY1, is a member of the largest metallopeptidase family called M20A. Aminoacylase-1 is a zinc-binding homodimeric enzyme expressed in kidney, brain, placenta and spleen. It is the most abundant of the aminoacylases. Defects in ACY1 are the cause of aminoacylase-1 deficiency (ACY1D). ACY1D results in a metabolic disorder manifesting with encephalopathy, unspecific psychomotor delay, psychomotor delay with atrophy of the vermis and syringomyelia, marked muscular hypotonia or normal clinical features. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids.
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NCBI登录号
- NP_000657
抗原
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