Glutarylcarnitine lithium

• CAT Number: I045602
• Molecular Formula: C12H20LiNO6
• Molecular Weight: 281.23
• Purity: ≥95%
• Synonyms: lithium;5-[(2R)-1-carboxylato-3-(trimethylazaniumyl)propan-2-yl]oxy-5-oxopentanoate
• InChI: InChI=1S/C12H21NO6.Li/c1-13(2,3)8-9(7-11(16)17)19-12(18)6-4-5-10(14)15;/h9H,4-8H2,1-3H3,(H-,14,15,16,17);/q;+1/p-1/t9-;/m1./s1
• InChIKey: FVZGWGXKHPPLDO-SBSPUUFOSA-M
• SMILES: [Li+].C[N+](C)(C)CC(CC(=O)[O-])OC(=O)CCCC(=O)[O-]

Glutarylcarnitine lithium is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes[1].
Malonylcarnitine and Glutarylcarnitine are important diagnostic metabolites in the screening of dried blood spots by tandem mass spectrometry[1].
The urinary excretion of glutarylcarnitine is a specific biochemical marker of glutaric acidemia type I (GA-1). The urinary excretion of glutarylcarnitine is an informative tool in the biochemical diagnosis of glutaric acidemia type I[2].

Reference

[1]. Johnson DW, et al. Stability of malonylcarnitine and Glutarylcarnitine in stored blood spots. J Inherit Metab Dis. 2004;27(6):789-90.
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[2]. S Tortorelli, et al. The urinary excretion of glutarylcarnitine is an informative tool in the biochemical diagnosis of glutaric acidemia type I. Mol Genet Metab. 2005 Feb;84(2):137-43.
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