推荐产品
产品名称
异丁酰基- L -肉碱, ≥97.0% (HPLC)
质量水平
方案
≥97.0% (HPLC)
表单
powder
旋光性
[α]/D -22±2°, c = 1 in H2O
杂质
≤10% water
颜色
white
储存温度
2-8°C
SMILES字符串
C[N+](C)(C)C[C@H](OC(C(C)C)=O)CC([O-])=O
InChI
1S/C11H21NO4/c1-8(2)11(15)16-9(6-10(13)14)7-12(3,4)5/h8-9H,6-7H2,1-5H3/t9-/m1/s1
InChI key
LRCNOZRCYBNMEP-SECBINFHSA-N
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生化/生理作用
异丁酰基-L-肉碱是酰基辅酶A脱氢酶（ACAD）的产物，酰基辅酶A脱氢酶是一组参与脂肪酸或支链氨基酸代谢的线粒体酶。
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
L Vallée et al.
European journal of pediatrics, 153(8), 598-603 (1994-08-01)
We describe on a 3-year-old child referred for evaluation and therapy of a cerebral vascular accident with residual hemiplegia and partial epilepsy. Metabolic investigations initially showed normal urinary organic acids as well as normal blood and urinary amino acids. Blood
N Sugiyama et al.
Acta paediatrica Japonica : Overseas edition, 32(4), 410-416 (1990-08-01)
A large quantity of propionylcarnitine in the urine of patients with propionic acidemia and methylmalonic aciduria was demonstrated. The amount excreted depended on the administered L-carnitine dose from 25 to 75 mg/kg/day. A high level of propionylcarnitine was also detected
M Fontaine et al.
Clinica chimica acta; international journal of clinical chemistry, 273(2), 161-170 (1998-07-10)
Here we report on a patient with severe ("non-classic") carnitine palmitoyltransferase type II (CPT II) deficiency. Hypoglycemia prompted by an infectious episode and associated with non-ketotic dicarboxylic aciduria orientated diagnosis towards beta-oxidation deficiency disorders. Blood carnitine levels revealed a secondary
Dwight D Koeberl et al.
Pediatric research, 54(2), 219-223 (2003-05-09)
Tandem mass spectrometry was adopted for newborn screening by North Carolina in April 1999. Since then, three infants with short-chain acyl-CoA dehydrogenase (SCAD) and one with isobutyryl-CoA dehydrogenase deficiency were detected on the basis of elevated butyrylcarnitine/isobutyrylcarnitine (C4-carnitine) concentrations in
K Matsumoto et al.
Clinica chimica acta; international journal of clinical chemistry, 216(1-2), 135-143 (1993-07-16)
An immobilized enzyme reactor, made up acylcarnitine hydrolase, carnitine dehydrogenase and diaphorase in sequence, was developed for the sensitive and selective determination of urinary free and individual acylcarnitines by a reversed-phase high-performance liquid chromatography. A 100-microliter urine sample was directly
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