Thus, 2,50-di(tertbutyl)-1,4,-benzohydroquinone (BHQ), a synthetic phenolic compound that inhibits sarcoendoplasmic Ca2+ ATPases (SERCAs) and with pro-oxidant properties, can also inhibit CaV2.1 voltage-dependent activation to ameliorate gating defects in the channel and subsequently prevent synaptic transmission problems produced by the S218L CACNA1A mutation causing a severe form of FHM with slowly progressive cerebellar ataxia and atrophy in humans [78]. The gene discussed is CACNA1A; the disease is aceruloplasminemia.