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Merck

50838

Sigma-Aldrich

R)-甲羟戊酸 锂盐

≥93.0% (qNMR)

别名:

R-MVA-Li, (R)-3,5-二羟基-3-甲基戊酸锂

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About This Item

经验公式(希尔记法):
C6H11LiO4
分子量:
154.09
MDL號碼:
分類程式碼代碼:
12352106
PubChem物質ID:
NACRES:
NA.25

化驗

≥93.0% (qNMR)

光學活性

[α]/D -26±3°, c = 0.1 in 0.1 M HCl (16 h)

儲存溫度

2-8°C

SMILES 字串

[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 密鑰

PVWNXFFXFNEHDZ-FYZOBXCZSA-M

應用

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

包裝

无底玻璃瓶。内含物装在插入的融合锥内。

象形圖

Exclamation mark

訊號詞

Warning

危險聲明

危險分類

Acute Tox. 4 Oral - Eye Irrit. 2

儲存類別代碼

11 - Combustible Solids

水污染物質分類(WGK)

WGK 3

閃點(°F)

Not applicable

閃點(°C)

Not applicable


历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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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
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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