51085
异丁酰基- L -肉碱
≥97.0% (TLC)
别名:
(2 R )-3-羧基- N ， N ， N -三甲基-2-（2-甲基-1-氧代丙氧基）-1-丙胺 内盐, L -肉碱异丁酰酯, iC4-肉碱
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关于此项目
经验公式（希尔记法）:
C11H21NO4
化学文摘社编号:
分子量:
231.29
NACRES:
NA.26
PubChem Substance ID:
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
4138517
产品名称
异丁酰基- L -肉碱, ≥97.0% (HPLC)
InChI key
LRCNOZRCYBNMEP-SECBINFHSA-N
SMILES string
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
assay
≥97.0% (HPLC)
form
powder
optical activity
[α]/D -22±2°, c = 1 in H2O
impurities
≤10% water
color
white
storage temp.
2-8°C
Quality Level
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Biochem/physiol Actions
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。
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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
D P Shalev et al.
Andrologia, 18(4), 368-375 (1986-07-01)
When washed human sperm were incubated in a modified Krebs-Ringer buffer solution in the absence of exogenous metabolizable substrates at 30 degrees C they maintained progressive motility for at least six hours. Under these conditions the spermatozoa apparently utilize endogenous
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
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