04725
3-Hydroxyglutaric acid
analytical standard
Sinonimo/i:
β-Hydroxyglutaric acid, 3-Hydroxypentanedioic acid
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About This Item
Formula empirica (notazione di Hill):
C5H8O5
Numero CAS:
Peso molecolare:
148.11
Beilstein:
1705476
Numero MDL:
Codice UNSPSC:
12352106
ID PubChem:
NACRES:
NA.24
Prodotti consigliati
Grado
analytical standard
Livello qualitativo
Saggio
≥95.0% (GC)
Durata
limited shelf life, expiry date on the label
applicazioni
clinical testing
Formato
neat
Temperatura di conservazione
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)
ZQHYXNSQOIDNTL-UHFFFAOYSA-N
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Azioni biochim/fisiol
3-Hydroxyglutaric acid is a glutaric acid derivative which is the byproduct of glutaric acidemia type I. 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. Studies indicate that 3-hydroxyglutaric acid can be used as biomarker for GCDH (glutaryl-CoA dehydrogenase) deficiency. It is believed that the excretion of 3-hydroxyglutaric acid is increased during ketosis, which occurs during glutaryl-CoA dehydrogenase deficiency. Studies on striatal cultures show that IGF-1 and FGF-2 (bFGF) reduces 3-hydroxyglutaric acid toxicity in striatal neurons.
Prodotti consigliati
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Codice della classe di stoccaggio
11 - Combustible Solids
Classe di pericolosità dell'acqua (WGK)
WGK 3
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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
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
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
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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