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Merck
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50405

Sigma-Aldrich

2-Methylbutyryl-L-carnitine

≥97.0% (HPLC)

Sinonimo/i:

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

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About This Item

Formula empirica (notazione di Hill):
C12H23NO4
Numero CAS:
256928-75-3
Peso molecolare:
245.32
Beilstein:
5946881
Numero MDL:
MFCD11975299
Codice UNSPSC:
41116107
ID PubChem:
329757597
NACRES:
NA.26

product name

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

Livello qualitativo

100

Saggio

≥97.0% (HPLC)

Forma fisica

powder or crystals

Attività ottica

[α]/D -20±2°, c = 0.1 in H2O

Impurezze

≤10% water

Colore

white to off-white

Temperatura di conservazione

2-8°C

Stringa SMILE

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

InChI

1S/C12H23NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3/t9?,10-/m1/s1
IHCPDBBYTYJYIL-QVDQXJPCSA-N

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Categorie correlate

Azioni biochim/fisiol

2-Methylbutyryl-L-carnitine is not usually detected in normal individuals. The elevation of 2-methylbutyrylcarnitine suggests a deficiency of a dehydrogenase specific for isobutyryl-CoA, important in the differential diagnosis of branched chain organic acidurias by analysis of urinary organic acids and acylcarnitines in plasma or dried blood spots.

Codice della classe di stoccaggio

11 - Combustible Solids

Classe di pericolosità dell'acqua (WGK)

WGK 3

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Certificati d'analisi (COA)

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K Kidouchi et al.
Clinica chimica acta; international journal of clinical chemistry, 173(3), 263-272 (1988-04-29)
A quantitative analysis for urinary acylcarnitines in a patient with neonatal multiple acyl-CoA dehydrogenation deficiency is described. This method (liquid chromatography) can quantify twelve acylcarnitines including glutarylcarnitine and 3 isomeric acylcarnitines (butyryl-1, valeryl- and octanoylisomer) in urine. Before and up
F Ferrara et al.
Drugs under experimental and clinical research, 31(3), 109-114 (2005-07-22)
The pool of different carnitine derivatives is formed by carnitine, acetylcarnitine, propionylcarnitine and isovalerylcarnitine. Isovalerylcarnitine is a compound performing activities that differ from those of the other carnitine esters. Its activity on proteolytic enzymes and on the calpain system has
Judit Bene et al.
World journal of gastroenterology, 12(1), 110-113 (2006-01-28)
To determine the plasma carnitine ester profile in adult patients with ulcerative culitis (UC) and compared with healthy control subjects. Using ESI triple quadrupole tandem mass spectrometry, the carnitine ester profile was measured in 44 patients with UC and 44
D S Millington et al.
The Journal of pediatrics, 110(1), 56-60 (1987-01-01)
A patient with isovaleryl-coenzyme A dehydrogenase deficiency was given a synthetic oral feed containing L-(2H3-methyl)-leucine of high isotopic purity as the only dietary precursor to the defective enzyme. Metabolites derived from this source were readily distinguished from their unlabeled endogenous
Prenatal diagnosis of isovaleric acidemia by fast atom bombardment and tandem mass spectrometry.
Y Shigematsu et al.
Clinica chimica acta; international journal of clinical chemistry, 203(2-3), 369-374 (1991-12-16)
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