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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 )
Catalog No. 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.
Purity : >98% (HPLC)
COA
Datasheet
HNMR
HPLC
MSDS
Handing Instructions
| Size | Price / USD | Stock | Quantity |
| 100MG | 37 | In Stock |
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| 200MG | Get Quote | In Stock |
|
| 500MG | Get Quote | In Stock |
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| 1G | Get Quote | In Stock |
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Biological Information
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Product Name2-Hydroxy-3-methylbutyric acid
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NoteResearch use only, not for human use.
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Brief Description2-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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Description2-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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In Vitro——
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In Vivo——
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Synonyms2-Hydroxy-3-methylbutanoic acid | (±)-2-Hydroxyisopentanoic Acid | 2-Hydroxyisovaleric Acid
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PathwayOthers
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TargetOther Targets
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RecptorOthers
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Research Area——
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Indication——
Chemical Information
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CAS Number4026-18-0
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Formula Weight118.13
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Molecular FormulaC5H10O3
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Purity>98% (HPLC)
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SolubilityDMSO:194.7 mM
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SMILESCC(C)C(O)C(O)=O
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Chemical Name——
Shipping & Storage Information
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Storage(-20℃)
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ShippingWith Ice Pack
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Stability≥ 2 years
Reference
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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