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Merck
모든 사진(1)

주요 문서

51371

Sigma-Aldrich

Isovaleryl-L-carnitine

≥94.0% (HPLC)

동의어(들):

(2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt, L-Carnitine isovaleryl ester, iC5-Carnitine

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About This Item

실험식(Hill 표기법):
C12H23NO4
CAS Number:
Molecular Weight:
245.32
Beilstein:
5946880
MDL number:
UNSPSC 코드:
12352209
PubChem Substance ID:
NACRES:
NA.26

Product Name

Isovaleryl-L-carnitine, ≥94.0% (HPLC)

Quality Level

분석

≥94.0% (HPLC)

양식

powder or crystals

광학 활성

[α]/D -23±2°, c = 1 in H2O

불순물

≤10% water

색상

white to off-white

저장 온도

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

InChI key

IGQBPDJNUXPEMT-SNVBAGLBSA-N

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애플리케이션


  • 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).

생화학적/생리학적 작용

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.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable


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