2-Methylbutyrylglycine (CAS 52320-67-9)
Alternate Names:
N-(2-Methyl-1-oxobutyl)glycine; N-(2-Methylbutyryl)glycine; N-sec-Valerylglycine
CAS Number:
52320-67-9
Molecular Weight:
159.18
Molecular Formula:
C7H13NO3
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
* Refer to Certificate of Analysis for lot specific data.
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2-Methylbutyrylglycine, or 2-MBG, is a metabolite generated in the body during the breakdown of specific amino acids. It falls under the acylglycines family and is derived from branched-chain amino acids. The detection of 2-Methylbutyrylglycine in urine samples can serve as an indicator of the presence of these disorders. 2-Methylbutyrylglycine influences the regulation of branched-chain amino acid metabolism. Some suggest that 2-Methylbutyrylglycine may act as an inhibitor of isovaleryl-CoA dehydrogenase, a key enzyme involved in the breakdown of isoleucine.
2-Methylbutyrylglycine (CAS 52320-67-9) References
- 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism. | Gibson, KM., et al. 2000. Pediatr Res. 47: 830-3. PMID: 10832746
- 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. | Korman, SH., et al. 2005. Clin Chem. 51: 610-7. PMID: 15615815
- Ethylmalonic encephalopathy: clinical and biochemical observations. | Zafeiriou, DI., et al. 2007. Neuropediatrics. 38: 78-82. PMID: 17712735
- 2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain. | Knebel, LA., et al. 2012. Brain Res. 1478: 74-82. PMID: 22967964
- Nontargeted LC-MS Metabolomics Approach for Metabolic Profiling of Plasma and Urine from Pigs Fed Branched Chain Amino Acids for Maximum Growth Performance. | Soumeh, EA., et al. 2016. J Proteome Res. 15: 4195-4207. PMID: 27704848
- Improved clinical outcome following liver transplant in patients with ethylmalonic encephalopathy. | Tam, A., et al. 2019. Am J Med Genet A. 179: 1015-1019. PMID: 30864297
- Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy. | Tein, I., et al. 1999. Neurology. 52: 366-72. PMID: 9932958
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
2-Methylbutyrylglycine, 250 mg | sc-482689 | 250 mg | $390.00 |