Figure 5

Glycine encephalopathy. Mutations in any one of three genes can cause Glycine encephalopathy (see MIM 605899 and disease group 542). All three potential pairwise interactions are found as predicted interactions in the OPHID database. No other database in iRefIndex includes these interactions. The three proteins are all part of the glycine decarboxylase complex; a loosely associated multienzyme complex consisting of four proteins that catalyzes the oxidative cleavage of glycine to carbon dioxide, ammonia, and a methylene group, in a multistep reaction. The fourth subunit (DLDH_HUMAN a.k.a. DLD or GCSL) has no interactions with any of the above three subunits in the iRefIndex. DLD is also a subunit of the branched-chain alpha-keto acid dehydrogenase complex (BCKD). Mutations in DLD or any other of the three catalytic subunits of this complex can lead to Maple Syrup Urine Disease (MIM 248600) - a disease with similar phenotype. This complex is not detected by any of the methods shown in this study since interactions between its subunits are not present in iRefIndex.