2-Hydroxy-3-methylbutanoic acid
| Code | Size | Price |
|---|
| TAR-T5273-100mg | 100mg | £103.00 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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Quantity:
Prices exclude any Taxes / VAT
Overview
Regulatory Status: RUO
Shipping:
cool pack
Storage:
-20℃
Documents
Further Information
Bioactivity:
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.
CAS:
4026-18-0
Formula:
C5H10O3
Molecular Weight:
118.132
Pathway:
; Metabolism
Purity:
0.98
SMILES:
CC(C)C(O)C(O)=O
Target:
Endogenous Metabolite
References
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.