4-Hydroxy-2-oxoglutarate aldolase inactivity in primary hyperoxaluria type 3 and glyoxylate reductase inhibition

► The PH3 HOGA mutations lead to unstable and inactive proteins. ► N-acetylneuraminate aldolase does not contribute to HOG cleavage. ► Glyoxylate reductase is inhibited by HOG. ► Glyoxylate reductase inhibition is one component of the molecular basis for PH3.