3-Methylcrotonylglycine, amino acid metabolite (ab145925)

Overview

  • Product name

    3-Methylcrotonylglycine, amino acid metabolite
  • Description

    Amino acid metabolite found in 3-methylcrotonylglycinuria
  • Alternative names

    • 2-(3-methylbut-2-enoylamino)acetic
    • 2-(3-methylbut-2-enoylamino)acetic acid
    • 3-Methylcrotonyl Glycine
    • 3MCG
    • beta-Methylcrotonylglycine
    • N-(3,3-Dimethylacrylyl)glycine
    • N-(3-Methyl-1-oxo-2-butenyl)glycine
    see all
  • Biological description

    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.

  • Purity

    > 95%
  • CAS Number

    33008-07-0
  • Chemical structure

    Chemical Structure

Properties

  • Chemical name

    2-(3-Methylbut-2-enoylamino)acetic acid
  • Molecular weight

    157.17
  • Molecular formula

    C7H11NO3
  • Storage instructions

    Store at -20°C. Store under desiccating conditions. The product can be stored for up to 12 months.
  • Handling

    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.

    Need more advice on solubility, usage and handling? Please visit our frequently asked questions (FAQ) page for more details.

  • Source

    Synthetic

References

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) »

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