所有图片(1)
About This Item
经验公式（希尔记法）:
C6H11LiO4
CAS号:
分子量:
154.09
MDL编号:
UNSPSC代码:
12352106
PubChem化学物质编号:
NACRES:
NA.25
方案:
≥93.0% (qNMR)
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方案
≥93.0% (qNMR)
旋光性
[α]/D -26±3°, c = 0.1 in 0.1 M HCl (16 h)
储存温度
2-8°C
SMILES字符串
[Li+].C[C@@](O)(CCO)CC([O-])=O
InChI
1S/C6H12O4.Li/c1-6(10,2-3-7)4-5(8)9;/h7,10H,2-4H2,1H3,(H,8,9);/q;+1/p-1/t6-;/m1./s1
InChI key
PVWNXFFXFNEHDZ-FYZOBXCZSA-M
应用
甲羟戊酸可用作分离或检测甲羟戊酸试验中的参考材料。甲羟戊酸是异戊烯焦磷酸（IPP）和甲羟戊酸途径的前体，它可以引导类异戊二烯生物的合成。甲羟戊酸可用作鉴定和表征甲羟戊酸激酶的底物。
生化/生理作用
甲羟戊酸是甲羟戊酸途径中的中间体，产生萜烯和类固醇。该功能提供治疗选择为常染色体隐性遗传甲羟戊酸尿症（一种胆固醇和非甾醇异戊二烯生物合成的先天性错误）患者的代谢紊乱 、R-甲羟戊酸累积提供了治疗选项。
包装
无底玻璃瓶。内含物装在插入的融合锥内。
警示用语：
Warning
危险声明
危险分类
Acute Tox. 4 Oral - Eye Irrit. 2
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
Mutational spectrum and genotype-phenotype correlations in mevalonate kinase deficiency.
Mandey, S. H., et al.
Human Mutation, 27, 796-802 (2006)
Discovery and chemistry of mevalonic acid.
Wagner, A. F. and Folkers, K.
Advances in Enzymology and Related Areas of Molecular Biology, 23, 471-483 (1961)
S M Houten et al.
Cellular and molecular life sciences : CMLS, 60(6), 1118-1134 (2003-07-16)
Mevalonate kinase (MK) is an essential enzyme in the isoprenoid biosynthesis pathway which produces numerous biomolecules (isoprenoids) involved in a variety of cellular processes. The indispensability of MK and isoprenoid biosynthesis for human health is demonstrated by the identification of
Irina Buhaescu et al.
Clinical biochemistry, 40(9-10), 575-584 (2007-05-01)
Mevalonate pathway is an important metabolic pathway which plays a key role in multiple cellular processes by synthesizing sterol isoprenoids, such as cholesterol, and non-sterol isoprenoids, such as dolichol, heme-A, isopentenyl tRNA and ubiquinone. While extensively studied in regard with
Dorothea Haas et al.
Orphanet journal of rare diseases, 1, 13-13 (2006-05-26)
Mevalonic aciduria (MVA) and hyperimmunoglobulinemia D syndrome (HIDS) represent the two ends of a clinical spectrum of disease caused by deficiency of mevalonate kinase (MVK), the first committed enzyme of cholesterol biosynthesis. At least 30 patients with MVA and 180
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