61076

DL-4-Hydroxy-2-keto-glutarsäure Lithiumsalz

analytical standard

• Synonym(e): 4-Hydroxy-2-oxo-glutarsäure Lithiumsalz, DL-KHG Li
• Empirische Formel (Hill-System): C₅H₆O₆ · xLi⁺
• CAS-Nummer: 1187-99-1
• Molekulargewicht: 162.10 (free acid basis)
• Beilstein: 1780096
• UNSPSC-Code: 41116107

Eigenschaften

• Qualität: analytical standard
• Assay: ≥97.0% (HPLC)
• Haltbarkeit: limited shelf life, expiry date on the label
• Anwendung(en): clinical testing
• Format: neat
• Lagertemp.: −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)
• InChIKey: WXSKVKPSMAHCSG-UHFFFAOYSA-N

Beschreibung

Allgemeine Beschreibung

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.

Anwendung

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).

Sicherheitshinweise

• Lagerklassenschlüssel: 11 - Combustible Solids
• WGK: WGK 3
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable