91298
3-羟基异戊酰-L-肉碱
analytical standard
别名:
（2R）-3-羧基-2-（3-羟基-3-甲基-1-氧代丁氧基]-N,N,N-三甲基-1-丙胺 内盐
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关于此项目
经验公式（希尔记法）:
C12H23NO5
化学文摘社编号:
分子量:
261.31
NACRES:
NA.24
UNSPSC Code:
41116107
MDL number:
Beilstein/REAXYS Number:
10549761
InChI
1S/C12H23NO5/c1-9(2)6-11(16)18-12(17,7-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3
SMILES string
C[N+](C)(C)C[C@H](OC(CC(C)(O)C)=O)CC([O-])=O
InChI key
DHRNMNHPSWBJEN-UHFFFAOYSA-N
grade
analytical standard
assay
≥90.0% (HPLC)
optical activity
[α]/D -17.0±2.0°, c = 0.1 in H2O
shelf life
limited shelf life, expiry date on the label
impurities
≤15% water (calc. on elemental analysis)
application(s)
clinical testing
format
neat
storage temp.
2-8°C
Quality Level
Biochem/physiol Actions
3-羟基异戊酰基-L-肉碱是一种内源性代谢产物，可能是亮氨酸分解代谢途径受损所致。在患有孤立的3-甲基巴豆酰-CoA羧化酶缺乏症的患者的血浆和尿液中观察到3-羟基异戊酰-L-肉碱，并将其用作生物素缺乏症在人和其他代谢性疾病中的生物标记物。
Other Notes
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存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
Urinary Excretion of 3-Hydroxyisovaleryl Carnitine Is an Early and Sensitive Indicator of Marginal Biotin Deficiency in Humans.
Stratton, S.L., et al.
The Journal of Nutrition, 141, 353-358 (2011)
3-Hydroxyisovalerylcarnitine in 3-methylcrotonyl-CoA carboxylase deficiency.
van Hove, J.L.K., et al.
Journal of inherited metabolic disease, 18, 592-601 (1995)
Quantitative Measurement of Urinary Excretion of 3-Hydroxyisovaleryl Carnitine by LC-MS/MS as an Indicator of Biotin Status in Humans.
Horvath, T.D., et al.
Analytical Chemistry, 82, 9543-9548 (2010)
Plasma concentration of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans.
Stratton, S.L., et al.
American Journal of Clinical Nutrition, 92, 1399-1405 (2010)
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