A Mouse Model of L-2-Hydroxyglutaric Aciduria, a Disorder of Metabolite Repair
Fig 4
Formation and breakdown of L-2-hydroxyglutarate and interference of this compound with lysine metabolism.
The scheme shows how L-2-hydroxyglutarate is formed and degraded. It also shows the initial steps of the major lysine catabolic pathway (via saccharopine) present in mammalian tissues and of the minor pathway (via L-pipecolate) present in brain and the inhibition exerted by L-2-hydroxyglutarate. 1. Lysine-α-ketoglutarate reductase; 2. Saccharopine dehydrogenase; 3. α-Aminoadipate semialdehyde dehydrogenase; 4. α-Aminoadipate transaminase; 5. Imine reductase; 6. L-Pipecolate oxidase.