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51371

Sigma-Aldrich

Isovaleryl-L-carnitine

≥94.0% (HPLC)

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Synonym(s):
(2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt, L-Carnitine isovaleryl ester, iC5-Carnitine
Empirical Formula (Hill Notation):
C12H23NO4
CAS Number:
Molecular Weight:
245.32
Beilstein/REAXYS Number:
5946880
PubChem Substance ID:
NACRES:
NA.26

Quality Level

assay

≥94.0% (HPLC)

form

powder or crystals

optical activity

[α]/D -23±2°, c = 1 in H2O

impurities

≤10% water

color

white to off-white

storage temp.

2-8°C

SMILES string

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

InChI key

IGQBPDJNUXPEMT-SNVBAGLBSA-N

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Show Differences

1 of 4

This Item
510854262350405
Isovaleryl-L-carnitine ≥94.0% (HPLC)

51371

Isovaleryl-L-carnitine

Isobutyryl-L-carnitine ≥97.0% (TLC)

51085

Isobutyryl-L-carnitine

Butyryl-L-carnitine ≥97.0% (TLC)

42623

Butyryl-L-carnitine

2-Methylbutyryl-L-carnitine ≥97.0% (HPLC)

50405

2-Methylbutyryl-L-carnitine

assay

≥94.0% (HPLC)

assay

≥97.0% (TLC)

assay

≥97.0% (TLC)

assay

≥97.0% (HPLC)

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

2-8°C

color

white to off-white

color

white

color

white to off-white

color

white to off-white

optical activity

[α]/D -23±2°, c = 1 in H2O

optical activity

[α]/D -22±2°, c = 1 in H2O

optical activity

[α]/D -23±2°, c = 1 in H2O

optical activity

[α]/D -20±2°, c = 0.1 in H2O

impurities

≤10% water

impurities

≤10% water

impurities

-

impurities

≤10% water

Biochem/physiol Actions

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.

Storage Class

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable


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Customers Also Viewed

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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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