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91298

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3-Hydroxyisovaleryl-L-carnitine

analytical standard

Synonyme(s) :

(2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt

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About This Item

Formule empirique (notation de Hill):
C12H23NO5
Numéro CAS:
Poids moléculaire :
261.31
Numéro Beilstein :
10549761
Code UNSPSC :
41116107
Nomenclature NACRES :
NA.24

Qualité

analytical standard

Niveau de qualité

Pureté

≥90.0% (HPLC)

Activité optique

[α]/D -17.0±2.0°, c = 0.1 in H2O

Durée de conservation

limited shelf life, expiry date on the label

Impuretés

≤15% water (calc. on elemental analysis)

Application(s)

clinical testing

Format

neat

Température de stockage

2-8°C

Chaîne SMILES 

C[N+](C)(C)C[C@H](OC(CC(C)(O)C)=O)CC([O-])=O

InChI

1S/C12H23NO5/c1-9(2)6-11(16)18-12(17,7-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3

Clé InChI

DHRNMNHPSWBJEN-UHFFFAOYSA-N

Actions biochimiques/physiologiques

3-Hydroxyisovaleryl-L-carnitine is an endogenous metabolite that can result from impairment in the leucine catabolic pathway. 3-Hydroxyisovaleryl-L-carnitine was observed in plasma and urine of patient with isolated 3-methylcrotonyl-CoA carboxylase deficiency and is used as biomarker of biotin deficiency in humans and other metabolic disorders.

Produits recommandés

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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

Urinary Excretion of 3-Hydroxyisovaleryl Carnitine Is an Early and Sensitive Indicator of Marginal Biotin Deficiency in Humans.
Stratton, S.L., et al.
The Journal of Nutrition, 141, 353-358 (2011)
Plasma concentration of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans.
Stratton, S.L., et al.
American Journal of Clinical Nutrition, 92, 1399-1405 (2010)
Quantitative Measurement of Urinary Excretion of 3-Hydroxyisovaleryl Carnitine by LC-MS/MS as an Indicator of Biotin Status in Humans.
Horvath, T.D., et al.
Analytical Chemistry, 82, 9543-9548 (2010)
3-Hydroxyisovalerylcarnitine in 3-methylcrotonyl-CoA carboxylase deficiency.
van Hove, J.L.K., et al.
Journal of inherited metabolic disease, 18, 592-601 (1995)

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