AGXT and primary hyperoxaluria type 1: Primary hyperoxaluria type 1 (PH1, MIM 259900), due to deficiency in peroxisomal alanine–glyoxylate aminotransferase (AGXT; Figure 1D), results in the accumulation of glyoxylate that is converted to insoluble oxalate and deposited in the kidney, leading to kidney stone formation and ultimately end-stage renal disease.