51371

Isovaleryl-L-carnitine

≥94.0% (HPLC)

• 別名: (2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt, L-Carnitine isovaleryl ester, iC5-Carnitine
• 実験式（ヒル表記法）: C₁₂H₂₃NO₄
• CAS番号: 31023-24-2
• 分子量: 245.32
• Beilstein: 5946880
• MDL番号: MFCD28580112
• UNSPSCコード: 12352209
• PubChem Substance ID: 329757684
• NACRES: NA.26

特性

• 製品名: Isovaleryl-L-carnitine, ≥94.0% (HPLC)
• 品質水準: 100
• アッセイ: ≥94.0% (HPLC)
• フォーム: powder or crystals
• 光学活性: [α]/D -23±2°, c = 1 in H₂O
• 不純物: ≤10% water
• 色: white to off-white
• 保管温度: 2-8°C
• SMILES記法: 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
• InChI Key: IGQBPDJNUXPEMT-SNVBAGLBSA-N

詳細

アプリケーション

• 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).

生物化学的／生理学的作用

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.

安全性情報

• 保管分類コード: 11 - Combustible Solids
• WGK: WGK 3
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable