It should be also mentioned that future potent and selective competitive CBS inhibitors could be used to improve folding and to stabilize active conformations of many pathogenic CBS mutant, where protein misfolding was identified or implied as a cause of loss of CBS activity and homocystinuria, and, perhaps paradoxically, they may in fact act as CBS activators or reactivators under such circumstances (as discussed in [188]). Here, CBS is linked to homocystinuria.