61076

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

analytical standard

• 同義詞: 4-Hydroxy-2-oxoglutaric acid lithium salt, DL-KHG Li
• 經驗公式（希爾表示法）: C₅H₆O₆ · xLi⁺
• CAS號碼: 1187-99-1
• 分子量：: 162.10 (free acid basis)
• Beilstein: 1780096
• 分類程式碼代碼: 41116107

屬性

• 等級: analytical standard
• 化驗: ≥97.0% (HPLC)
• 儲存期限: limited shelf life, expiry date on the label
• 應用: clinical testing
• 格式: neat
• 儲存溫度: −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 密鑰: WXSKVKPSMAHCSG-UHFFFAOYSA-N

描述

一般說明

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.

應用

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).

安全資訊

• 儲存類別代碼: 13 - Non Combustible Solids
• 水污染物質分類（WGK）: WGK 3
• 閃點（°C）: Not applicable