51371
异戊酰-L -肉碱
≥94.0% (HPLC)
别名:
(2 R )-3-羧基- N ， N ， N -三甲基-2-（3-甲基-1-氧代丁氧基）-1-丙胺 内盐, L-肉碱异戊酸酯, iC5-肉碱
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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
MDL number:
UNSPSC Code:
12352209
PubChem Substance ID:
NACRES:
NA.26
Beilstein/REAXYS Number:
5946880
产品名称
异戊酰-L -肉碱, ≥94.0% (HPLC)
SMILES string
C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O
InChI key
IGQBPDJNUXPEMT-SNVBAGLBSA-N
InChI
1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3/t10-/m1/s1
assay
≥94.0% (HPLC)
form
powder or crystals
optical activity
[α]/D -23±2°, c = 1 in H2O
impurities
≤10% water
color
white to off-white
storage temp.
2-8°C
Quality Level
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Application
- L：-富马酸肉碱和异戊酰-L：-富马酸左旋肉碱和富马酸异戊酰左旋肉碱可加速妊娠小鼠实验性骨质疏松症后骨体积/总体积比的恢复。：该研究探讨了异戊酰基-L-肉碱在改善骨质疏松症患者骨量方面的治疗潜力，表明其对骨骼健康具有重大影响（Patano et al., 2008）。
- 左旋肉碱和富马酸异戊酰左旋肉碱对人体成骨细胞的体外增殖和分化有积极影响。：本文讨论了异戊酰-L-肉碱对人类成骨细胞形成和健康的积极影响，展示了其在再生医学和骨骼修复方面的应用（Colucci et al., 2005）。
Biochem/physiol Actions
异戊酰肉碱是异戊酸血症的表型异常，由异戊酸的积累引起，对中枢神经系统有毒。异戊酸血症是由线粒体酶异戊酰辅酶A脱氢酶缺乏引起的亮氨酸代谢的常染色体隐性先天性错缺陷，导致异戊酰辅酶A衍生物的积累。它是人类中第一个公认的有机酸血症，可导致显著的发病率和死亡率。早期诊断和蛋白限制饮食以及补充肉毒碱和甘氨酸的治疗可有效促进严重受影响个体的正常发育。通过甘氨酸-N-酰化酶的另一种途径允许通过产生可被排泄的异戊酰-甘氨酸进行解毒治疗。因此，异戊酰肉碱和异戊酰甘氨酸分别是血浆和尿液中该疾病的标志，并且无论患者′的代谢状况如何，其水平均升高。
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
Jerry Vockley et al.
American journal of medical genetics. Part C, Seminars in medical genetics, 142C(2), 95-103 (2006-04-08)
Isovaleric acidemia (IVA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans
Organic acid profile of isovaleric acidemia: a comprehensive metabolomics approach.
Dercksen, M., et al.
Metabolomics, 9, 765-777 (2013)
F Salamino et al.
Pediatric research, 36(2), 182-186 (1994-08-01)
When a 12-y-old girl suffering from isovaleric acidemia was treated with L-carnitine, there was a considerable increase in her blood and urine concentration of isovalerylcarnitine. When later the patient received an infusion of glycine in place of carnitine, isovalerylcarnitine reverted
David Meierhofer
PloS one, 14(8), e0221342-e0221342 (2019-08-16)
Acylcarnitines are fatty acyl esters of L-carnitine and facilitate the entry of long-chain fatty acids into mitochondria via the carnitine shuttle, where they are metabolized via ß-oxidation. Alterations of acylcarnitine species can be diagnostic for fatty acid oxidation disorders and
M F Silva et al.
Clinical biochemistry, 34(8), 635-638 (2002-02-19)
The effect of administration of the antiepileptic drug valproate (VPA), on the composition of the plasma acylcarnitine profile (including free carnitine) was investigated. Plasma samples were obtained from 18 individuals (13 males:5 females; 15-65 y) on long-term treatment with VPA
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