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
请在我们的在线平台NMR用请在我们的在线平台NMR用ChemisTwin®查找本产品的数字化标准物质。您可以利用ChemisTwin®上的数字化等效物质进行样品确认和化合物定量（采用数字化外标）。您可以查看该物质的NMR 谱，点击鼠标即可与您的样品在线比较。点击此处了解更多信息，并开始免费试用。
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存储类别
11 - Combustible Solids
wgk
WGK 3
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
Paris Jafari et al.
PloS one, 8(1), e53735-e53735 (2013-01-18)
Glutaric aciduria type I (glutaryl-CoA dehydrogenase deficiency) is an inborn error of metabolism that usually manifests in infancy by an acute encephalopathic crisis and often results in permanent motor handicap. Biochemical hallmarks of this disease are elevated levels of glutarate
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 |