Product name3-Methylcrotonylglycine, amino acid metabolite
DescriptionAmino acid metabolite found in 3-methylcrotonylglycinuria
- 2-(3-methylbut-2-enoylamino)acetic acid
- 3-Methylcrotonyl Glycine
Amino acid metabolite found in the metabolic disorder 3-methylcrotonylglycinuria. It is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. Increased levels of this metabolite are found in patients suffering from leucine catabolic disorders, such as 3-methylcrotonyl-CoA carboxylase deficiency and it is often considered a diagnostic marker of organic acidemias. Disrupts mitochondrial energy homeostasis and inhibits synaptic Na+, K+-ATPase activity in brain of young rats.
Chemical name2-(3-Methylbut-2-enoylamino)acetic acid
Storage instructionsStore at -20°C. Store under desiccating conditions. The product can be stored for up to 12 months.
Wherever possible, you should prepare and use solutions on the same day. However, if you need to make up stock solutions in advance, we recommend that you store the solution as aliquots in tightly sealed vials at -20°C. Generally, these will be useable for up to one month. Before use, and prior to opening the vial we recommend that you allow your product to equilibrate to room temperature for at least 1 hour.
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This product has been referenced in:
- Moura AP et al. 3-Methylcrotonylglycine disrupts mitochondrial energy homeostasis and inhibits synaptic Na(+),K (+)-ATPase activity in brain of young rats. Cell Mol Neurobiol 32:297-307 (2012). Read more (PubMed: 21993987) »
- Gallardo ME et al. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am J Hum Genet 68:334-46 (2001). Read more (PubMed: 11170888) »