3-Hydroxyglutaric acid
SIAL/04725 - analytical standard
Synonym: β-Hydroxyglutaric acid; 3-
CAS Number: 638-18-6
Empirical Formula (Hill Notation): C5H8O5
Molecular Weight: 148.11
MDL Number: MFCD06208235
Linear Formula: C5H8O5
Product Type: Chemical
| application(s) | clinical testing |
| assay | ≥95.0% (GC) |
| format | neat |
| grade | analytical standard |
| InChI | 1S/C5H8O5/c6-3(1-4(7)8)2- |
| InChI key | ZQHYXNSQOIDNTL-UHFFFAOYSA |
| Quality Level | 100  |
| shelf life | limited shelf life, expiry date on the label |
| storage temp. | 2-8°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. |
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| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 3 |
| Purity | ≥95.0% (GC) |
| Storage Temp. | 2-8°C |
| UNSPSC | 12352106 |