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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
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产品名称2-Hydroxy-3-methylbutyric acid
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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
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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.
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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.
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同义词2-Hydroxy-3-methylbutanoic acid;(±)-2-Hydroxyisopentanoic Acid;2-Hydroxyisovaleric Acid
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通路Others
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靶点Other Targets
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受体Others
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研究领域——
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适应症——
化学信息
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CAS Number4026-18-0
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分子量118.13
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分子式C5H10O3
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纯度>98% (HPLC)
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溶解度DMSO:194.7 mM
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SMILESCC(C)C(O)C(O)=O
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化学全称——
运输与储存
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储存条件(-20℃)
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运输条件With Ice Pack
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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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