Abstract
Thiamine (thiamin, vitamin B1) is an essential nutrient for animals. Thiamine is incorporated into cells and is then rapidly converted to thiamine pyrophosphate that works as a coenzyme in several enzymes in glucose and amino acid metabolism. It is known that thiamine deficiency leads to neuronal dysfunction of the peripheral nervous system (beriberi) and focal lesions in particular brain regions (Wernicke-Korsakoff syndrome, WKS). Especially, pathogenesis of WKS has attracted considerable attention because of the region-specific vulnerability, which is also observed in neurodegenerative diseases. Several underlying mechanisms (excitotoxicity, oxidative stress, disruption of blood brain barrier, endoplasmic reticulum stress, astroglial dysfunction) have been reported to be involved in the pathogenesis of WKS by using rodent models of thiamine deficiency. However, the precise mechanisms causing selective topographic vulnerability in the brains of WKS and the rodent model still remain unclear. In this review, I provide an update on function of thiamine, and describe about thiamine deficiency syndromes, especially WKS. Furthermore, I discuss possible mechanisms involved in pathogenesis of focal brain lesions in experimental thiamine deficiency based on the data available in the literature and from our laboratory.
