Accéder au contenu
Merck
Toutes les photos(1)

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

Se connecterpour consulter vos tarifs contractuels et ceux de votre entreprise/organisme


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

Vous recherchez des produits similaires ? Visite Guide de comparaison des produits

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


Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

Déjà en possession de ce produit ?

Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.

Consulter la Bibliothèque de documents

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
Organic acid profile of isovaleric acidemia: a comprehensive metabolomics approach.
Dercksen, M., et al.
Metabolomics, 9, 765-777 (2013)
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

Notre équipe de scientifiques dispose d'une expérience dans tous les secteurs de la recherche, notamment en sciences de la vie, science des matériaux, synthèse chimique, chromatographie, analyse et dans de nombreux autres domaines..

Contacter notre Service technique