Product: | 3-Hydroxyglutaric Acid | ||||||||
---|---|---|---|---|---|---|---|---|---|
Catalog Number: | 5175 | ||||||||
CAS Number: | 638-18-6 | ||||||||
Synonyms: |
3-Hydroxy-pentanedioic Acid; 2,4-Dideoxypentaric Acid; β-Hydroxyglutaric Acid |
||||||||
Pricing: |
|
||||||||
Formula: | C5H8O5 | ||||||||
Molecular Weight: | 148.11 | ||||||||
Structure: | |||||||||
Appearance: | Off-White to Light Pink Solid | ||||||||
Category: | Reference Standards for Inborn Errors | ||||||||
Stability: | Stable under recommended storage conditions. | ||||||||
Storage: |
Keep tightly closed. |
||||||||
Transportation: |
Non-hazardous for transport |
||||||||
Literature References: |
Bjugstad, K.B., et al. 2001. J. Inherit. Metab. Dis. 24: 631-647. PMID: 11768583; Pitt, J., et al. 2002. J. Inherit. Metab. Dis. 25: 83-88. PMID: 12118531; Ferreira, G.C., et al. 2007. Int. J. Dev. Neurosci. 25: 391-398. PMID: 17643899 |
||||||||
MSDS: | |||||||||
Description: |
3-Hydroxyglutaric Acid is a glutaric acid derivative which is the byproduct of Glutaric acidemia type I. Studies indicate that 3-Hydroxyglutaric Acid can be used as a marker 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. |