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04725

Supelco

3-Hydroxyglutaric acid

analytical standard

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Synonym(s):
β-Hydroxyglutaric acid, 3-Hydroxypentanedioic acid
Empirical Formula (Hill Notation):
C5H8O5
CAS Number:
638-18-6
Molecular Weight:
148.11
Beilstein:
1705476
MDL number:
MFCD06208235
PubChem Substance ID:
329747978
NACRES:
NA.24

grade

analytical standard

Quality Level

100

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

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This Item
54925C68539H3898
3-Hydroxyglutaric acid analytical standard

Supelco

04725

3-Hydroxyglutaric acid

(S)-3-Hydroxybutyric acid ≥97.0%

Sigma-Aldrich

54925

(S)-3-Hydroxybutyric acid

3-Chloro-2,2-dimethylpropionic acid 99%

Sigma-Aldrich

C68539

3-Chloro-2,2-dimethylpropionic acid

(±)-3-Hydroxyoctanoic acid ≥97%

Sigma-Aldrich

H3898

(±)-3-Hydroxyoctanoic acid

format

neat

format

-

format

-

format

-

assay

≥95.0% (GC)

assay

≥97.0%

assay

99%

assay

≥97%

shelf life

limited shelf life, expiry date on the label

shelf life

-

shelf life

-

shelf life

-

application(s)

clinical testing

application(s)

-

application(s)

-

application(s)

-

storage temp.

2-8°C

storage temp.

2-8°C

storage temp.

-

storage temp.

−20°C

Biochem/physiol Actions

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.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

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Sigma-Aldrich

116807

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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
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
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
Panagiotis Georgios Kanellopoulos et al.
Journal of environmental sciences (China), 99, 222-238 (2020-11-14)
PM10 samples were collected from an urban/industrial site nearby Athens, where uncontrolled burning activities occur. PAHs, monocarboxylic, dicarboxylic, hydroxycarboxylic and aromatic acids, tracers from BVOC oxidation, biomass burning tracers and bisphenol A were determined. PAH, monocarboxylic acids, biomass burning tracers
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