50892
辛酰- L -肉碱
≥97.0% (TLC)
别名:
(2R)-3-羧基-N,N,N-三甲基-2-[（1-氧代辛基）氧]-1-丙胺 内盐, 左旋-肉碱辛酸酯, C8-肉碱
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关于此项目
经验公式（希尔记法）:
C15H29NO4
化学文摘社编号:
分子量:
287.40
MDL number:
UNSPSC Code:
12352211
PubChem Substance ID:
NACRES:
NA.26
Beilstein/REAXYS Number:
5955761
产品名称
辛酰- L -肉碱, ≥97.0% (TLC)
SMILES string
C[N+](C)(C)C[C@H](OC(CCCCCCC)=O)CC([O-])=O
InChI
1S/C15H29NO4/c1-5-6-7-8-9-10-15(19)20-13(11-14(17)18)12-16(2,3)4/h13H,5-12H2,1-4H3/t13-/m1/s1
InChI key
CXTATJFJDMJMIY-CYBMUJFWSA-N
assay
≥97.0% (TLC)
optical activity
[α]/D -17±2°, c = 0.1 in H2O
impurities
≤10% water
storage temp.
2-8°C
Quality Level
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Biochem/physiol Actions
L-辛酰基肉碱是辛酰基肉碱的生理活性形式，在中链酰基辅酶 a 脱氢酶 (MCAD) 缺乏症中检测到。MCAD 表现为不耐受长时间禁食，反复发作低血糖昏迷伴中链二羧酸尿症，酮体生成障碍，血浆和组织肉碱水平低下。
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
David S Millington et al.
Methods in molecular biology (Clifton, N.J.), 708, 55-72 (2011-01-06)
The acylcarnitine profile is a diagnostic test for inherited disorders of fatty acid and branched-chain amino acid catabolism. Patients with this type of metabolic disorder accumulate disease-specific acylcarnitines that correlate with the acyl coenzyme A compounds in the affected mitochondrial
Steen Larsen et al.
The Journal of physiology, 590(14), 3349-3360 (2012-05-16)
Skeletal muscle mitochondrial content varies extensively between human subjects. Biochemical measures of mitochondrial proteins, enzyme activities and lipids are often used as markers of mitochondrial content and muscle oxidative capacity (OXPHOS). The purpose of this study was to determine how
J Will Thompson et al.
Rapid communications in mass spectrometry : RCM, 26(21), 2548-2554 (2012-09-26)
Acylcarnitines are routinely analyzed by electrospray ionization tandem mass spectrometry (ESI-MS/MS) both in clinical diagnostic and public health newborn screening laboratories from plasma and dried whole blood spots (DBS) on filter paper. The use of DBS as a convenient method
Josiane Joseph et al.
Metabolites, 8(4) (2018-10-05)
Duchenne muscular dystrophy (DMD) is a musculoskeletal disorder that causes severe morbidity and reduced lifespan. Individuals with DMD have an X-linked mutation that impairs their ability to produce functional dystrophin protein in muscle. No cure exists for this disease and
Judit Bene et al.
World journal of gastroenterology, 11(42), 6671-6675 (2006-01-21)
To determine the fasting plasma carnitine ester in patients with celiac disease. We determined the fasting plasma carnitine ester profile using ESI triple quadrupol mass spectrometry in 33 adult patients with biopsy-confirmed maturity onset celiac disease maintained on long term
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