Molecular Requirements for Peroxisomal Targeting of Alanine-Glyoxylate Aminotransferase as an Essential Determinant in Primary Hyperoxaluria Type 1

摘要

The crystal structure of the peroxisome enzyme alanine-glyoxylate aminotransferase bound to its targeting receptor Pex5p explains why even minor fold defects prevent targeting of the enzyme and cause kidney disease.