SLC19A3 and biotin-responsive basal ganglia disease: Biotine-responsive basal ganglia disease (BBGD)/ SLC19A3 deficiency: thiamine pyrophosphate (TPP) is an important cofactor of more steps in energy production playing main role in several pathways in neurometabolism and SLC19A3 is a specific biotin-dependent thiamine transporter in basal ganglia system [57–59].