Thiamine pyrophosphate (TPP) is a coenzyme for pyruvate dehydrogenase, α-ketoglutarate dehydrogenase and transketolase. The first two of these enzymes function in the metabolism of carbohydrates, while transketolase functions in the pentose phosphate pathway to synthesize NADPH and the pentose sugarsdeoxyribose and ribose. Systemic thiamine deficiency can lead to myriad problems including neurodegeneration, wasting, and death. Well-known syndromes caused by lack of thiamine due to malnutrition or a diet high in thiaminase-rich foods include Wernicke-Korsakoff syndrome and beriberi, diseases also common in chronic abusers of alcohol.
Thiamine pyrophosphate (TPP)
Genetic diseases of thiamine transport are rare but serious. Thiamine Responsive Megaloblastic Anemia with diabetes mellitus and sensorineural deafness (TRMA) (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=249270) is an autosomal recessive disorder caused by mutations in the gene SLC19A2 (http://www.ncbi.nlm.nih.gov:80/entrez/dispomim.cgi?id=603941), a high affinity thiamine transporter. TRMA patients do not show signs of systemic thiamine deficiency, suggesting redundancy in the thiamine transport system. This has led to the discovery of a second high affinity thiamine transporter, SLC19A3 (http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=606152).
VitaminB1 is key for proper muscle tone of the intestines, stomach, and heart.
VitaminB1 can be taken postoperatively for pain from dental procedures, to relieve air or seasickness and in the treatment of herpes zoster.
Congenital defects: Maple syrup disease, congenital lactate acidosis, subacute necrotizing encephalopathy, and vitamin B1-responsive megaloblastic anemia are inherited diseases with congenital defects in vitaminB1 metabolism.