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
Beilstein:
5946881
MDL编号:
UNSPSC代码:
41116107
PubChem化学物质编号:
NACRES:
NA.26
产品名称
2-Methylbutyryl-L-carnitine, ≥97.0% (HPLC)
质量水平
方案
≥97.0% (HPLC)
表单
powder or crystals
旋光性
[α]/D -20±2°, c = 0.1 in H2O
杂质
≤10% water
颜色
white to off-white
储存温度
2-8°C
SMILES字符串
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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生化/生理作用
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.
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
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
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
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
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)
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
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