As reviewed,11,14 about half of genetically tested UFS individuals carry biallelic variants in HPSE2 (OMIM UFS2 #236730), which encodes heparanase-2, a protein that binds heparan sulfate and also inhibits the endoglycosidase enzymatic activity of a related protein called heparanase.15 This evidence concerns the gene HPSE2 and Ochoa syndrome.