Review Article

Metabolic Causes of Epileptic Encephalopathy

Figure 1

Cobalamin transport and metabolism. Hydroxycobalamin (OH-Cbl) enters the cell bound to transcobalamin (TC), a binding protein. The hydroxycobalamin-transcobalamin complex is broken down inside the lysosome, and the enzyme cobalamin C (CblC) removes the hydroxyl group to generate free cobalamin (Cbl), which is synthesized via additional steps into methyl- and adenosyl-cobalamin.
124934.fig.001