For research use only. Not for therapeutic Use.
3-Methylglutarylcarnitine Chloride(CAT: C001121) is a specialized acylcarnitine derivative involved in mitochondrial fatty acid metabolism and the transport of fatty acids into the mitochondria for β-oxidation. It is formed from the conjugation of 3-methylglutaric acid with carnitine and plays a role in energy production, particularly in tissues with high energy demands like the heart and skeletal muscles. This compound is significant in metabolic research and is used as a biomarker for diagnosing organic acidurias, such as 3-methylglutaconic aciduria, a disorder related to mitochondrial dysfunction. It is valuable in studying metabolic diseases and mitochondrial disorders.
CAS Number | 821794-55-2 |
Synonyms | 3-carboxy-2-(4-carboxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium Chloride |
Molecular Formula | C₁₃H₂₄ClNO₆ |
Purity | ≥95% |
Solubility | Methanol (Slightly), Water (Slightly) |
Appearance | White to Off-White Solid |
Storage | -20°C, Hygroscopic |
IUPAC Name | [3-carboxy-2-(4-carboxy-3-methylbutanoyl)oxypropyl]-trimethylazanium;chloride |
InChI | InChI=1S/C13H23NO6.ClH/c1-9(5-11(15)16)6-13(19)20-10(7-12(17)18)8-14(2,3)4;/h9-10H,5-8H2,1-4H3,(H-,15,16,17,18);1H |
InChIKey | AUYDMXVJGDGHRP-UHFFFAOYSA-N |
SMILES | CC(CC(=O)O)CC(=O)OC(CC(=O)O)C[N+](C)(C)C.[Cl-] |