50405
2-Methylbutyryl-L-carnitine
≥97.0% (HPLC)
别名:
(2R)-3-Carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-1-propanaminium inner salt
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关于此项目
经验公式（希尔记法）:
C12H23NO4
化学文摘社编号:
分子量:
245.32
NACRES:
NA.26
PubChem Substance ID:
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
5946881
产品名称
2-Methylbutyryl-L-carnitine, ≥97.0% (HPLC)
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
SMILES string
C[N+](C)(C)C[C@H](OC(C(CC)C)=O)CC([O-])=O
InChI key
IHCPDBBYTYJYIL-QVDQXJPCSA-N
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
Quality Level
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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.
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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
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
J E Abdenur et al.
Journal of inherited metabolic disease, 21(6), 624-630 (1998-10-08)
Tandem mass spectrometric analysis of acylcarnitines and amino acids has been applied in newborn screening programmes for the detection of several inborn errors of metabolism. We report a false positive result for isovaleric acidaemia in a newborn screening programme using
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
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
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