For research use only. Not for therapeutic Use.
Glutarylcarnitine lithium (Cat No.: I045602) is the lithium salt form of glutarylcarnitine, an acylcarnitine derived from glutaric acid and carnitine. It is an important biomarker for glutaric acidemia type I (GA1), an inherited metabolic disorder caused by glutaryl-CoA dehydrogenase deficiency. Measurement of glutarylcarnitine in blood or urine is widely used in newborn screening and metabolic diagnostics. The lithium salt form enhances solubility and stability, making it suitable for research applications in metabolomics, clinical biochemistry, and the study of fatty acid and amino acid catabolism.
Synonyms | lithium;5-[(2R)-1-carboxylato-3-(trimethylazaniumyl)propan-2-yl]oxy-5-oxopentanoate |
Molecular Formula | C12H20LiNO6 |
Purity | ≥95% |
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-] |
Reference | [1]. Johnson DW, et al. Stability of malonylcarnitine and Glutarylcarnitine in stored blood spots. J Inherit Metab Dis. 2004;27(6):789-90. [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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