Glutarylcarnitine

• CAT Number: M004901
• CAS Number: 102636-82-8
• Molecular Formula: C12H21NO6
• Molecular Weight: 275.30
• Purity: ≥95%
• Synonyms: (3R)-3-(4-carboxybutanoyloxy)-4-(trimethylazaniumyl)butanoate
• InChI: InChI=1S/C12H21NO6/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)/t9-/m1/s1
• InChIKey: NXJAXUYOQLTISD-SECBINFHSA-N
• SMILES: C[N+](C)(C)CC(CC(=O)[O-])OC(=O)CCCC(=O)O

Glutarylcarnitine is the diagnostic metabolite for malonic aciduria and glutaric aciduria type I monitored in most tandem mass spectrometry newborn screening programmes.
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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