51371

Isovaleryl-L-carnitin

≥94.0% (HPLC)

• Synonym(e): (2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium Inneres Salz, L-Carnitin-isovalerylester
• Empirische Formel (Hill-System): C₁₂H₂₃NO₄
• CAS-Nummer: 31023-24-2
• Molekulargewicht: 245.32
• Beilstein: 5946880
• MDL-Nummer: MFCD28580112
• UNSPSC-Code: 12352209
• PubChem Substanz-ID: 329757684
• NACRES: NA.26

Eigenschaften

• Produktbezeichnung: Isovaleryl-L-carnitin, ≥94.0% (HPLC)
• Qualitätsniveau: 100
• Assay: ≥94.0% (HPLC)
• Form: powder or crystals
• Optische Aktivität: [α]/D -23±2°, c = 1 in H₂O
• Verunreinigungen: ≤10% water
• Farbe: white to off-white
• Lagertemp.: 2-8°C
• SMILES String: C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O
• 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
• InChIKey: IGQBPDJNUXPEMT-SNVBAGLBSA-N

Beschreibung

Anwendung

• 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. Wirkung

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.

Sicherheitshinweise

• Lagerklassenschlüssel: 11 - Combustible Solids
• WGK: WGK 3
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable