ASPH and facial dysmorphism-lens dislocation-anterior segment abnormalities-spontaneous filtering blebs syndrome: The observation that none of the tested AspH variants catalyzes substantial levels of substrate-uncoupled 2-oxoacid turnover in vitro (Fig. S4), as is the case for wt AspH (52), implies that the Traboulsi syndrome–associated substitutions may not exert pathogenicity by directly regulating (local) metabolism in cells, including in a manner related to substrate-coupled and -uncoupled turnover of 2OG as proposed for FIH (62, 100, 101, 102).