Marked reduction of Na+, K+-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice

► A knockout model of glutaric aciduria type I (Gcdh−/−) was supplemented with lysine. ► Creatine kinase activity was inhibited in Gcdh−/− mice after lysine supplementation. ► Na+, K+- ATPase activity was inhibited in Gcdh−/− mice after lysine supplementation. ► Acute lysine administration disturbed the cellular bioenergetics in Gcdh−/− mice.