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DL-3-AMINOISOBUTYRIC ACID

CAS No. 144-90-1

DL-3-AMINOISOBUTYRIC ACID ( 3-Amino-2-methylpropanoic acid | α-Methyl-β-alanine )

产品货号. M19753 CAS No. 144-90-1

β-氨基异丁酸是 N-氨甲酰-β-氨基异丁酸被β-脲基丙酸酶 (EC 3.5.1.6) 转化的产物,这是嘧啶降解的最后一步。 β-脲基丙酸酶缺乏症是与神经系统异常相关的嘧啶降解的先天性错误。

纯度: >98% (HPLC)

COA Datasheet HNMR HPLC MSDS Handing Instructions
规格 价格/人民币 库存 数量
100MG ¥332 有现货
200MG 获取报价 有现货
500MG 获取报价 有现货
1G 获取报价 有现货

生物学信息

  • 产品名称
    DL-3-AMINOISOBUTYRIC ACID
  • 注意事项
    本公司产品仅用于科研实验,不得用于人体或动物的临床与诊断
  • 产品简述
    β-氨基异丁酸是 N-氨甲酰-β-氨基异丁酸被β-脲基丙酸酶 (EC 3.5.1.6) 转化的产物,这是嘧啶降解的最后一步。 β-脲基丙酸酶缺乏症是与神经系统异常相关的嘧啶降解的先天性错误。
  • 产品描述
    beta-aminoisobutyric acid is the product from the conversion of N-carbamyl-beta-aminoisobutyric acid by the enzyme Beta-ureidopropionase (EC 3.5.1.6) the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities.
  • 体外实验
    ——
  • 体内实验
    Weight is slightly decreased in the mice by the end of 3-Amino-2-methylpropanoic acid (BAIBA) treatment. Analysis of body composition using MRI demonstrates BAIBA treatment significantly decreases body fat in the mice. Consistent with the effects on thermogenic and β-oxidation gene expression and body weights, analysis with metabolic cages indicates that oxygen consumption (VO2) and whole body energy expenditure are increased in the BAIBA treated mice without any significant difference in activity or food intake. The mice are also challenged with an intraperitoneal glucose tolerance test (IPGTT). BAIBA is found to significantly improve the glucose tolerance in the mice as determined by the area under the curve of the IPGTT.
  • 同义词
    3-Amino-2-methylpropanoic acid | α-Methyl-β-alanine
  • 通路
    Proteasome/Ubiquitin
  • 靶点
    Endogenous Metabolite
  • 受体
    Human Endogenous Metabolite
  • 研究领域
    ——
  • 适应症
    ——

化学信息

  • CAS Number
    144-90-1
  • 分子量
    103.12
  • 分子式
    C4H9NO2
  • 纯度
    >98% (HPLC)
  • 溶解度
    DMSO:10 mM
  • SMILES
    CC(CN)C(O)=O
  • 化学全称
    ——

运输与储存

  • 储存条件
    (-20℃)
  • 运输条件
    With Ice Pack
  • 稳定性
    ≥ 2 years

参考文献

1.Kuilenburg A B P V Meinsma R Beke E et al. beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities.[J]. Human Molecular Genetics 2004 13(22):2793-2801.
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