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Key Documents

51371

Sigma-Aldrich

Isovaleryl-L-carnitine

≥94.0% (HPLC)

Synonyme(s) :

(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

Formule empirique (notation de Hill):
C12H23NO4
Numéro CAS:
Poids moléculaire :
245.32
Numéro Beilstein :
5946880
Code UNSPSC :
12352209
ID de substance PubChem :
Nomenclature NACRES :
NA.26

product name

Isovaleryl-L-carnitine, ≥94.0% (HPLC)

Niveau de qualité

Pureté

≥94.0% (HPLC)

Forme

powder or crystals

Activité optique

[α]/D -23±2°, c = 1 in H2O

Impuretés

≤10% water

Couleur

white to off-white

Température de stockage

2-8°C

Chaîne SMILES 

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

Clé InChI

IGQBPDJNUXPEMT-SNVBAGLBSA-N

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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).

Actions biochimiques/physiologiques

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.

Code de la classe de stockage

11 - Combustible Solids

Classe de danger pour l'eau (WGK)

WGK 3

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable


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Consulter la Bibliothèque de documents

Organic acid profile of isovaleric acidemia: a comprehensive metabolomics approach.
Dercksen, M., et al.
Metabolomics, 9, 765-777 (2013)
Jerry Vockley et al.
American journal of medical genetics. Part C, Seminars in medical genetics, 142C(2), 95-103 (2006-04-08)
Isovaleric acidemia (IVA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans
F Salamino et al.
Pediatric research, 36(2), 182-186 (1994-08-01)
When a 12-y-old girl suffering from isovaleric acidemia was treated with L-carnitine, there was a considerable increase in her blood and urine concentration of isovalerylcarnitine. When later the patient received an infusion of glycine in place of carnitine, isovalerylcarnitine reverted
M H Fries et al.
The Journal of pediatrics, 129(3), 449-452 (1996-09-01)
To assess the effectiveness of glycine and carnitine therapy on isovaleryl conjugate excretion in isovaleric acidemia (IVA). Urinary isovalerylglycine (IVG) and isovalerylcarnitine (IVC) were measured from 12-hour urine specimens collected overnight from an 8-year-old patient with IVA (who had no
David Meierhofer
PloS one, 14(8), e0221342-e0221342 (2019-08-16)
Acylcarnitines are fatty acyl esters of L-carnitine and facilitate the entry of long-chain fatty acids into mitochondria via the carnitine shuttle, where they are metabolized via ß-oxidation. Alterations of acylcarnitine species can be diagnostic for fatty acid oxidation disorders and

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