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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:
12352200
PubChem Substance ID:
329757597
NACRES:
NA.26

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
S Pontremoli et al.
Biochemical and biophysical research communications, 148(3), 1189-1195 (1987-11-13)
Isovalerylcarnitine (IVC) a product of the catabolism of L-leucine, is a potent activator of the Ca2+-dependent proteinase (calpain) of human neutrophils. At concentrations of Ca2+ in the low micromolar range, activation was 12 to 15-fold, and the activity exceeded that
M Fontaine et al.
Clinica chimica acta; international journal of clinical chemistry, 252(2), 109-122 (1996-08-30)
Carnitine levels and acylcarnitine profiles in a patient with mild multiple acyl-CoA dehydrogenase deficient beta-oxidation were compared with control results. Whereas blood and urine total carnitine levels were moderately decreased, blood esterified carnitine levels in the patient were about 2-fold
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
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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