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50405

Sigma-Aldrich

2-Methylbutyryl-L-carnitine

≥97.0% (HPLC)

Synonym(s):

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

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About This Item

Empirical Formula (Hill Notation):
C12H23NO4
CAS Number:
256928-75-3
Molecular Weight:
245.32
Beilstein:
5946881
MDL number:
MFCD11975299
UNSPSC Code:
41116107
PubChem Substance ID:
329757597
NACRES:
NA.26

product name

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

Quality Level

100

Assay

≥97.0% (HPLC)

form

powder or crystals

optical activity

[α]/D -20±2°, c = 0.1 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(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

InChI key

IHCPDBBYTYJYIL-QVDQXJPCSA-N

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Biochem/physiol Actions

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.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Dietrich Matern et al.
Pediatrics, 112(1 Pt 1), 74-78 (2003-07-03)
2-methylbutyryl-CoA dehydrogenase deficiency, also known as short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency, is a recently described autosomal recessive disorder of L-isoleucine metabolism. Only 4 affected individuals in 2 families have been described. One patient developed athetoid cerebral palsy, and another had
R A Chalmers et al.
Pediatric research, 18(12), 1325-1328 (1984-12-01)
Concentrations of l-carnitine and acylcarnitines have been determined in urine from patients with disorders of organic acid metabolism associated with an intramitochondrial accumulation of acyl-CoA intermediates. These included propionic acidemia, methylmalonic aciduria, isovaleric acidemia, multicarboxylase deficiency, 3-hydroxy-3-methylglutaric aciduria, methylacetoacetyl-CoA thiolase
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
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
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