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首页- Products - Others - Other Targets - 2-Hydroxy-3-methylbutyric acid

2-Hydroxy-3-methylbutyric acid

CAS No. 4026-18-0

2-Hydroxy-3-methylbutyric acid ( 2-Hydroxy-3-methylbutanoic acid;(±)-2-Hydroxyisopentanoic Acid;2-Hydroxyisovaleric Acid )

产品货号. M19995 CAS No. 4026-18-0

2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.

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
    2-Hydroxy-3-methylbutyric 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
    2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.
  • 产品描述
    2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.
  • 同义词
    2-Hydroxy-3-methylbutanoic acid;(±)-2-Hydroxyisopentanoic Acid;2-Hydroxyisovaleric Acid
  • 通路
    Others
  • 靶点
    Other Targets
  • 受体
    Others
  • 研究领域
    ——
  • 适应症
    ——

化学信息

  • CAS Number
    4026-18-0
  • 分子量
    118.13
  • 分子式
    C5H10O3
  • 纯度
    >98% (HPLC)
  • 溶解度
    DMSO:194.7 mM
  • SMILES
    CC(C)C(O)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.Magni F et al. Simultaneous determination of plasma levels of alpha-ketoisocaproic acid and leucine and evaluation of alpha-[1-13C]ketoisocaproic acid and [1-13C]leucine enrichment by gas chromatography-mass spectrometry. Anal Biochem. 1994 Aug 1;220(2):308-14.
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