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
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
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
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)
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
G Hoffmann et al.
The New England journal of medicine, 314(25), 1610-1614 (1986-06-19)
A two-year-old boy presented with severe failure to thrive, developmental delay, anemia, hepatosplenomegaly, central cataracts, and dysmorphic features. Quantitative analyses of urinary organic acids revealed massive excretion of mevalonic acid, a metabolic precursor of cholesterol and nonsterol isoprenes: 46,000 to
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