DL-4-Hydroxy-2-ketoglutarate is one of the metabolites that is characteristically elevated in the urine of patients with type 3 primary hyperoxaluria (PH3), and it is produced by the accumulation of substrates of mitochondrial 4-hydroxy-2-oxoglutarate aldolase ( HOGA ). DL-4-Hydroxy-2-ketoglutarate can be used in studies related to PH3.
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