50405
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:
Molecular Weight:
245.32
NACRES:
NA.26
PubChem Substance ID:
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
5946881
Product Name
2-Methylbutyryl-L-carnitine, ≥97.0% (HPLC)
Quality Level
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
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.
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Storage Class
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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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