51371

Sigma-Aldrich

Isovaleryl-L-carnitine

≥94.0% (HPLC)

Synonym(s):
L-Carnitine isovaleryl ester, iC5-Carnitine, (2R)-3-Carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-1-propanaminium inner salt
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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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.

RIDADR

NONH for all modes of transport

WGK Germany

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificate of Analysis

Certificate of Origin

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 F Silva et al.
Clinical biochemistry, 34(8), 635-638 (2002-02-19)
The effect of administration of the antiepileptic drug valproate (VPA), on the composition of the plasma acylcarnitine profile (including free carnitine) was investigated. Plasma samples were obtained from 18 individuals (13 males:5 females; 15-65 y) on long-term treatment with VPA...
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...

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