所有图片(1)
别名:
β-羟基戊二酸, 3-羟基戊二酸
经验公式（希尔记法）:
C5H8O5
CAS号:
分子量:
148.11
Beilstein:
1705476
MDL编号:
UNSPSC代码:
12352106
PubChem化学物质编号:
NACRES:
NA.24
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等级
analytical standard
质量水平
检测方案
≥95.0% (GC)
保质期
limited shelf life, expiry date on the label
应用
clinical testing
格式
neat
储存温度
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
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相关类别
生化/生理作用
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。
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WGK
WGK 3
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
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
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
J Pitt et al.
Journal of inherited metabolic disease, 25(2), 83-88 (2002-07-18)
Three patients with ketosis had increased excretion of 3-hydroxyglutarate (21.8-37.9 micromol/mmol creatinine; controls 2.3 +/- 1.6), an indicator of glutaryl-CoA dehydrogenase deficiency (GDHD), which normalized when the patients were nonketotic. Clinical assessment of all three patients and enzyme studies in
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