Type-1 PH (PH1) results from genetic mutations of the AGXT gene, which encodes the hepatic peroxisomal enzyme, alanine:glyoxylate-aminotransferase (AGT). AGXT is the only gene known to encode AGT and, thus far, approximately 146 mutations have been associated with PH1 [2–4]. The gene discussed is AGT; the disease is primary hyperoxaluria type 1.