61076

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

analytical standard

• Sinónimos: 4-Hydroxy-2-oxoglutaric acid lithium salt, DL-KHG Li
• Fórmula empírica (notación de Hill): C₅H₆O₆ · xLi⁺
• Número de CAS: 1187-99-1
• Peso molecular: 162.10 (free acid basis)
• Beilstein/REAXYS Number: 1780096
• UNSPSC Code: 41116107

Propiedades

• 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

Descripción

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).

Información de seguridad

• Storage Class: 11 - Combustible Solids
• wgk_germany: WGK 3
• flash_point_f: Not applicable
• flash_point_c: Not applicable