61076

DL-4-Hydroxy-2-ketoglutaric acid lithium salt

analytical standard

• Synonym(s): 4-Hydroxy-2-oxoglutaric acid lithium salt, DL-KHG Li
• Empirical Formula (Hill Notation): C₅H₆O₆ · xLi⁺
• CAS Number: 1187-99-1
• Molecular Weight: 162.10 (free acid basis)
• Beilstein: 1780096
• UNSPSC Code: 41116107

Properties

• grade: analytical standard
• Assay: ≥97.0% (HPLC)
• shelf life: limited shelf life, expiry date on the label
• application(s): clinical testing
• format: neat
• storage temp.: −20°C
• InChI: 1S/C5H6O6/c6-2(4(8)9)1-3(7)5(10)11/h2,6H,1H2,(H,8,9)(H,10,11)
• InChI key: WXSKVKPSMAHCSG-UHFFFAOYSA-N

Description

General description

DL-4-Hydroxy-2-ketoglutaric acid lithium salt (HOG) is a precursor substrate of 4-hydroxy-2-oxoglutarate aldolase (HOGA1), a unique enzyme in the hydroxyproline catabolism pathway. It is reported that the primary hyperoxaluria type 3, which is a recently discovered form of the autosomal recessive disease, results from the mutations in the gene coding for HOGA1.

Application

DL-4-Hydroxy-2-ketoglutaric acid lithium salt may be used as an analytical reference standard for the determination of the analyte in animal biological samples using ion chromatography coupled to negative electrospray mass spectrometry (IC/ES-MS). This can be used to determine the primary hyperoxalurias PH Type 3 (PH3), caused by the mutations in the mitochondrial enzyme, 4-hydroxy-2-oxoglutarate aldolase (HOGA).

Safety Information

• Storage Class Code: 13 - Non Combustible Solids
• WGK: WGK 3
• Flash Point(C): Not applicable