Kinetic studies with selected 2OG derivatives and hydrophobic 2-oxoacids bearing a single carboxylate group were performed using SPE–MS to quantify their efficiency in sustaining catalysis of the Traboulsi syndrome–associated AspH variants and to rank their therapeutic potential (Table 4, Table S4). Here, ASPH is linked to facial dysmorphism-lens dislocation-anterior segment abnormalities-spontaneous filtering blebs syndrome.