The EIF2B2 gene, located on chromosome 14q24.3, encodes the beta subunit of the eIF2B protein. EIF2B interacts with the eIF2 protein to regulate protein synthesis in the cell, and it catalyzes the exchanges of GDP and GTP for its activation and deactivation. Defects in this protein have been associated with leukodystrophy with vanishing white matter (VWM), characterized by spasticity, progressive cerebellar ataxia, and inconstant optic atrophy.