(C5) Isovalerylcarnitine
| Cat. # | Quantity | Price | Lead time | Buy this product |
|---|---|---|---|---|
| 4324-5mg | 5 mg | please inquire | 7 days |
(C5) Isovalerylcarnitine is an analogue of endogenous acylcarnitine, formed in the body as a result of leucine metabolism, and has an isovaleric acid residue as an acyl substituent. The profile of acylcarnitines in blood and tissues is used as a marker of various conditions associated with mitochondrial (and/or peroxisomal) dysfunction.
The product is used primarily as a control for MS/MS.
Isovalerylcarnitine — the main biochemical marker for isovaleric acidemia, which occurs as a result of deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase. Isovalerylcarnitine has been found to be associated with celiac disease and very long chain acyl-CoA dehydrogenase deficiency (VLCAD). Also, the level of isovalerylcarnitine is determined when studying neuralgia, for example, with trigeminal neuralgia.
General properties
| Appearance: | white solid |
| Molecular weight: | 281.78 |
| CAS number: | 31023-24-2 (inner salt); 139144-12-0 (chloride) |
| Molecular formula: | C12H24ClNO4 |
| IUPAC name: | (2R)-3-Carboxy-N,N,N-trimethyl-2-[(3-methylbutanoyl)oxy]-1-propanaminium |
| Solubility: | DMF, DMSO, ethanol |
| Quality control: | NMR 1H and HPLC-MS (95+%) |
| Storage conditions: | 24 months after receival at -20°C in the dark. Transportation: at room temperature for up to 3 weeks. Desiccate. |
| MSDS: | Download |
| Product specifications |
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