Primary hyperoxaluria type I (PH1) is a rare kidney diseasedueto the deficit of alanine:glyoxylate aminotransferase (AGT), a pyridoxal-5′-phosphate-dependentenzyme responsible for liver glyoxylate detoxification, which in turnprevents oxalate formation and precipitation as kidney stones. This evidence concerns the gene AGXT and primary hyperoxaluria type 1.