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
CAS Number:
分子量:
245.32
Beilstein:
5946880
MDL编号:
UNSPSC代码:
12352209
PubChem化学物质编号:
NACRES:
NA.26
Product Name
异戊酰-L -肉碱, ≥94.0% (HPLC)
质量水平
方案
≥94.0% (HPLC)
表单
powder or crystals
旋光性
[α]/D -23±2°, c = 1 in H2O
杂质
≤10% water
颜色
white to off-white
储存温度
2-8°C
SMILES字符串
C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O
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
InChI key
IGQBPDJNUXPEMT-SNVBAGLBSA-N
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应用
- 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）。
生化/生理作用
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。
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
Organic acid profile of isovaleric acidemia: a comprehensive metabolomics approach.
Dercksen, M., et al.
Metabolomics, 9, 765-777 (2013)
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
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
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
M H Fries et al.
The Journal of pediatrics, 129(3), 449-452 (1996-09-01)
To assess the effectiveness of glycine and carnitine therapy on isovaleryl conjugate excretion in isovaleric acidemia (IVA). Urinary isovalerylglycine (IVG) and isovalerylcarnitine (IVC) were measured from 12-hour urine specimens collected overnight from an 8-year-old patient with IVA (who had no
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