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Supelco

3-Hydroxyisovaleryl-L-carnitine

analytical standard

Synonym(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

Empirical Formula (Hill Notation):
C12H23NO5
CAS Number:
99159-87-2
Molecular Weight:
261.31
Beilstein:
10549761
UNSPSC Code:
41116107
NACRES:
NA.24

grade

analytical standard

Quality Level

100

Assay

≥90.0% (HPLC)

optical activity

[α]/D -17.0±2.0°, c = 0.1 in H2O

shelf life

limited shelf life, expiry date on the label

impurities

≤15% water (calc. on elemental analysis)

application(s)

clinical testing

format

neat

storage temp.

2-8°C

SMILES string

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

InChI key

DHRNMNHPSWBJEN-UHFFFAOYSA-N

Related Categories

Biochem/physiol Actions

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.

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WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

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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)
3-Hydroxyisovalerylcarnitine in 3-methylcrotonyl-CoA carboxylase deficiency.
van Hove, J.L.K., et al.
Journal of inherited metabolic disease, 18, 592-601 (1995)
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)
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)

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