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91298

Supelco

3-Hydroxyisovaleryl-L-carnitine

analytical standard

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Synonym(s):
(2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt
Empirical Formula (Hill Notation):
C12H23NO5
CAS Number:
Molecular Weight:
261.31
Beilstein/REAXYS Number:
10549761

grade

analytical standard

Quality Level

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

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This Item
542534985352096
3-Hydroxyisovaleryl-L-carnitine analytical standard

91298

3-Hydroxyisovaleryl-L-carnitine

grade

analytical standard

grade

analytical standard

grade

analytical standard

grade

analytical standard

assay

≥90.0% (HPLC)

assay

≥97.0% (HPLC)

assay

≥95.0% (LC/MS)

assay

≥95.0% (TLC)

optical activity

[α]/D -17.0±2.0°, c = 0.1 in H2O

optical activity

[α]/D -28.0±2.0°, c = 0.1 in H2O

optical activity

[α]/D -20±3°, c = 0.1 in methanol

optical activity

[α]/D -20.5±3.5°, c = 0.1 in methanol

shelf life

limited shelf life, expiry date on the label

shelf life

limited shelf life, expiry date on the label

shelf life

limited shelf life, expiry date on the label

shelf life

limited shelf life, expiry date on the label

impurities

≤15% water (calc. on elemental analysis)

impurities

≤15% water (calculated from elemental analysis)

impurities

-

impurities

-

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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Storage Class

11 - Combustible Solids

wgk_germany

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)
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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