Since NADH dehydrogenase deficiency in LS impairs the oxidation of NADH to NAD+ causing decreased NAD+ (particularly in mitochondria), the metabolic profiles of LS brain and hearts are mainly characterised by derangement of several NAD+‐dependent metabolic enzymes. The gene discussed is ENSG00000254051; the disease is hyperinsulinemic hypoglycemia, familial, 4.