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Merck

36105

Sigma-Aldrich

(±)β-羟基异丁酸钠

≥96.0%

别名:

β-羟基异丁酸 钠盐, (±)-β-HIBA-Na, 3-羟基-2-甲基丙酸 钠盐, 3-羟基-2-甲基丙酸钠

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About This Item

经验公式(希尔记法):
C4H7NaO3
分子量:
126.09
MDL號碼:
分類程式碼代碼:
12352106
PubChem物質ID:
NACRES:
NA.25

品質等級

化驗

≥96.0% (GC)
≥96.0%

形狀

powder

成份

sodium, 17.5-19.0%

儲存溫度

2-8°C

SMILES 字串

[Na+].CC(CO)C([O-])=O

InChI

1S/C4H8O3.Na/c1-3(2-5)4(6)7;/h3,5H,2H2,1H3,(H,6,7);/q;+1/p-1

InChI 密鑰

RBJZIQZDAZLXEK-UHFFFAOYSA-M

應用

3-羟基异丁酸(HIBA)可用于研究参与支链氨基酸代谢的酶的分布、表征和动力学,例如,3-羟基异丁酸脱氢酶(EC:1.1.1.31)和3-羟基异丁酰辅酶A水解酶(EC:3.1.2.4)。

生化/生理作用

3-羟基异丁酸(HIBA)在缬氨酸代谢中形成,是3-羟基异丁酸脱氢酶(EC: 1.1.1.31)和3-羟基异丁酰辅酶A水解酶(EC:3.1.2.4)的反应物;在多发性硬化(MS)患者代谢谱中浓度升高

包裝

无底玻璃瓶。内含物装在锥底内插管中。

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


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Disorders of the metabolism of amino acids and related compounds.
Shih, V. E. and Mandell, R. et al.
Genetic Disorders and the Fetus, 514-553 (2010)
Mammalian 3-hydroxyisobutyrate dehydrogenase.
J W Hawes et al.
Methods in enzymology, 324, 218-228 (2000-09-16)
Norbert W Lutz et al.
Biochemical and biophysical research communications, 354(1), 160-164 (2007-01-16)
(1)H NMR spectroscopy of cerebrospinal fluid (CSF) is currently being used to study metabolic profiles characteristic of distinct multiple sclerosis (MS) manifestations. For select MS patient groups, we have previously detected significantly increased concentrations of several identified metabolites and one
Ference J Loupatty et al.
American journal of human genetics, 80(1), 195-199 (2006-12-13)
Only a single patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency has been described in the literature, and the molecular basis of this inborn error of valine catabolism has remained unknown until now. Here, we present a second patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency
Radovan Murín et al.
Journal of neurochemistry, 105(4), 1176-1186 (2008-02-21)
The branched-chain amino acids (BCAAs)--isoleucine, leucine, and valine--belong to the limited group of substances transported through the blood-brain barrier. One of the functions they are thought to have in brain is to serve as substrates for meeting parenchymal energy demands.

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