04725
3-羟基戊二酸
analytical standard
别名:
β-羟基戊二酸, 3-羟基戊二酸
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关于此项目
经验公式（希尔记法）:
C5H8O5
化学文摘社编号:
分子量:
148.11
NACRES:
NA.24
PubChem Substance ID:
UNSPSC Code:
12352106
MDL number:
Beilstein/REAXYS Number:
1705476
grade
analytical standard
Quality Level
assay
≥95.0% (GC)
shelf life
limited shelf life, expiry date on the label
application(s)
clinical testing
format
neat
storage temp.
2-8°C
InChI
1S/C5H8O5/c6-3(1-4(7)8)2-5(9)10/h3,6H,1-2H2,(H,7,8)(H,9,10)
InChI key
ZQHYXNSQOIDNTL-UHFFFAOYSA-N
Biochem/physiol Actions
3-羟基戊二酸是一种戊二酸衍生物，是戊二酸血症I型的副产物。戊二酸尿症I型（戊二酰辅酶A脱氢酶缺乏症）是一种先天性代谢病，通常在婴儿期表现为急性脑病危象，常导致永久性运动障碍。研究表明，3-羟基戊二酸可作为GCDH（戊二酰辅酶A脱氢酶）缺乏症的生物标志物。有人认为，在酮症期间，3-羟基戊二酸的分泌量增加，这是戊二酰辅酶A脱氢酶缺乏症期间会发生的。对纹状体培养物的研究表明，IGF-1和FGF-2（bFGF）可降低纹状体神经元中3-羟基戊二酸的毒性。
Other Notes
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存储类别
11 - Combustible Solids
wgk
WGK 3
Gustavo C Ferreira et al.
International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 25(6), 391-398 (2007-07-24)
Glutaric acidemia type I is an inherited metabolic disorder caused by a severe deficiency of the mitochondrial glutaryl-CoA dehydrogenase activity leading to accumulation of predominantly glutaric and 3-hydroxyglutaric acids in the brain tissue of the affected patients. Considering that a
K B Bjugstad et al.
Journal of inherited metabolic disease, 24(6), 631-647 (2002-01-05)
Glutaric acid (GA) and 3-hydroxyglutaric acid (3GA) are thought to contribute to the degeneration of the caudate and putamen that is seen in some children with glutaric acidaemia type I, a metabolic disorder caused by a glutaryl-CoA dehydrogenase deficiency. This
Garfield A Simon et al.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 1097-1098, 101-110 (2018-09-16)
Glutaric aciduria type 1, a deficiency of glutaryl-CoA dehydrogenase, causes an accumulation of neurotoxic metabolites glutaric acid and 3-hydroxyglutaric acid (3-HGA). Testing of these analytes is routinely done by GC-MS but seldom account for interference from isomers or compounds with
全球贸易项目编号
| 货号 | GTIN |
|---|---|
| 04725-10MG | 04061838635709 |
| 04725-50MG | 04061838635716 |