Thiamine levels in the blood of homozygous KO and KI mice fed a conventional diet are decreased to 0.058±0.051 and 0.126±0.092 μM, respectively, at 7 weeks compare to WT mice (0.796±0.259 μM). When WT and homozygous KO and KI mice are fed a Thiamine-restricted diet (Thiamine: 0.60 mg/100 g food), blood Thiamine concentration at 5 and 14 days is markedly decreased to 0.010±0.009 and 0.010±0.006 μM, respectively, compare to WT mice (0.609±0.288 μM). Thiamine concentration in brain homogenate of WT mice fed a conventional diet is 3.81±2.18 nmol/g wet weight, and that of KO and KI is 1.33±0.96 and 2.16±1.55 nmol/g wet weight, respectively. Notably, Thiamine concentration in brain homogenate decreases steadily in KO and KI mice fed a thiamine-restrict diet (Thiamine: 0.60 mg/100 g food) for 5 days (0.95±0.72 nmol/g wet weight) and 14 days (1.11±0.24 nmol/g wet weight), respectively, compare to WT (3.65±1.02 nmol/g wet weight), before the mice presenting an phenotype of disease.