36105
(±)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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关于此项目
经验公式（希尔记法）:
C4H7NaO3
化学文摘社编号:
分子量:
126.09
NACRES:
NA.25
PubChem Substance ID:
UNSPSC Code:
12352106
MDL number:
InChI
1S/C4H8O3.Na/c1-3(2-5)4(6)7;/h3,5H,2H2,1H3,(H,6,7);/q;+1/p-1
SMILES string
[Na+].CC(CO)C([O-])=O
InChI key
RBJZIQZDAZLXEK-UHFFFAOYSA-M
assay
≥96.0% (GC), ≥96.0%
form
powder
composition
sodium, 17.5-19.0%
storage temp.
2-8°C
Quality Level
Application
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）。
Biochem/physiol Actions
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。
Packaging
无底玻璃瓶。内含物装在锥底内插管中。
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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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