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Normal L2HGDH protein docked to its substrate L-2-hydroxyglutarate molecule

Interaction studies for L2HGDH and its substrate L-2-hydroxyglutarate predicted five amino acids (ie, Gln-89, Tyr-195, Val-404, Ala-402, and Gly-403) of wild-type L2HGDH are involved in the interaction with its substrate via conventional hydrogen bonding. All these binding sites are within the FAD-dependent enzyme domain. (Source: Muzammal M, Ali MZ, Brugger B, et al. A novel protein-truncating mutation in L2HGDH causes L-2-hydroxyglutaric aciduria in a consanguineous Pakistani family. Metab Brain Dis 2022;37[1]:243-52. Creative Commons Attribution 4.0 License, https://creativecommons.org/licenses/by/4.0.)

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