Synonym: (2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt; L-Carnitine isovaleryl ester; iC5-Carnitine
CAS Number: 31023-24-2
Empirical Formula (Hill Notation): C12H23NO4
Molecular Weight: 245.32
MDL Number: MFCD28580112
Linear Formula: C12H23NO4
Product Type: Chemical
| assay |
≥94.0% (HPLC) |
| color |
white to off-white |
| form |
powder or crystals |
| impurities |
≤10% water |
| InChI |
1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3/t10-/m1/s1 |
| InChI key |
IGQBPDJNUXPEMT-SNVBAGLBSA-N |
| optical activity |
[α]/D -23±2°, c = 1 in H2O |
| Quality Level |
100  |
| SMILES string |
C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O |
| storage temp. |
2-8°C |
| Application: |
- L: -carnitine fumarate and isovaleryl-L: -carnitine fumarate accelerate the recovery of bone volume/total volume ratio after experimentally induced osteoporosis in pregnant mice.: This study explores the therapeutic potential of Isovaleryl-L-carnitine in improving bone volume in conditions of osteoporosis, indicating its significant impact on bone health (Patano et al., 2008
 ).
- L-carnitine and isovaleryl L-carnitine fumarate positively affect human osteoblast proliferation and differentiation in vitro.: This article discusses the positive effects of Isovaleryl-L-carnitine on human osteoblast cells, contributing to bone formation and health, showcasing its applications in regenerative medicine and bone repair (Colucci et al., 2005 ).
|
| Biochem/physiol Actions: |
Isovalerylcarnitine is the phenotypic abnormality in iso-valeric acidemia resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase allows detoxification by producing isovaleryl-glycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patient′s metabolic condition. |
| RIDADR |
NONH for all modes of transport |
| WGK Germany |
WGK 3 |
| Flash Point(F) |
Not applicable |
| Flash Point(C) |
Not applicable |
| Purity |
≥94.0% (HPLC) |
| Storage Temp. |
2-8°C |
| UNSPSC |
12352209 |
