It consists of five subunits, α to ε, encoded by EIF2B1-EIF2B5 genes, all of which are linked to a severe inherited human neurodegenerative disorder called Leukoencephalopathy with Vanishing White Matter, or VWM (for review, see [79]). The gene discussed is EIF2B1; the disease is leukoencephalopathy with vanishing white matter.