INTR ODUCTI ON.... . . ......... . .. . . . . . . . ....... 483 HIST ORY OF THI AMIN . ..... . . . . . . . ... . .. .. . . . . . . . . . . . . . . . . . . . . . . .... . . . . . . . . . . . . . . .. ... . . . . . . . . . 484 BI OCHEMISTRY AN D MET AB OLISM 485 Biosynthesis . . . . . . . . . ..... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 485 Biochemical Role of TDP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487 B ALAN CE ..... . . .... ... . . .. . . . .. . . . . . . . . . . . . . .. 488 Brain Content. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488 Absorption . . . . . . . . . . . . . . . . . . . . .... . . . . . . . . . . . . . . ... . .... . . . . . . . . . . . . . . . . . . . .... . . . . . . . . . . . . . . .. .. . . . 489 Blood-Brain Barrier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489 Antithiamins. . . . . .. .. . . . . . . . . . . . . . . . . . . . . . . . . . ...... . .. . . . . . . . . . . . . . . . . .... . . . . . . . . . . . . . . . .. ... . . . . . 490 Evaluation of Deficiency .. . . . . . . . . . . . . . .. .. . . . . . . . . . . . . . . . . . ...... .. . . . . . . . . . . . ........ .. . . . . . . . 491 THI AMIN IN CENTR AL NERV OUS SYSTEM FUN CTI ON . . . . . . . . . . .. . . . ...... . . . . . . . . . 492 Energy Metabolism . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . 492 Pentose Phosphate Pathway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 494 Neurotransmitters . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 495 Role in Nerve Conduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 497 Biosynthetic Role. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . 498 Animal Models of Thiamin Deficiency . . . . . .. . . . . . . . . . . ... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 499 THI AMIN IN H UM AN DISEASES. ........ ... . . . 500 Subclinical Deficiency . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 500 Beriberi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 501 Wernicke-Korsakoff Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 503 Subacute Necrotizing Encephalomyelopathy . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . 504 Th�am�n-Resp'o�sive Maple Syrup Urine Disease. . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505 Thlamm TOxicity. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 506 CON CLUSI ON . ......... . . . . . . . ....... ...... ... . . . . .. 506

Thiamin and the brain.

IN 1969 Rogers et al. described a case of unexplained anemia in association with diabetes mellitus and sensorineural deafness.1 The anemia responded only to pharmacologic closes of thiamine. Anothe...

Thiamine-Dependent Beriberi in the “Thiamine-Responsive Anemia Syndrome”

Thiamine-responsive anemia in DIDMOAD syndrome

Thiamine-responsive megaloblastic anemia, sensorineural deafness, and diabetes mellitus: A new syndrome?

Diabetes mellitus, thiamine-dependentmegaloblastic anemia, and sensorineural deafness associated with deficient α-ketoglutarate dehydrogenase activity

The mechanism of glucose intolerance in thiamine-deficient rats has been examined. Deficient rats showed marked glucose intolerance. However, the hypoglycaemic effect of insulin (1 i.u. kg−1, i.p.) was similar in the deficient, pair-fed and normal groups, though somewhat weaker in the normal group than in the other two groups. After injection of tolbutamide (40 mg kg−1, i.p.), the hypoglycaemic effects in the three groups were the same. Tyramine (10 mg kg−1, s.c.) restored the impaired glucose tolerance of deficient rats to normal, but not that of alloxan diabetic rats. Furthermore, tyramine did not restore the intolerance of deficient rats pretreated with alloxan. These results suggest that the main factor causing glucose intolerance in the deficient rats may be suppressed insulin secretion.

T. Nishikawa

Papers

Glucose intolerance in thiamine-deficient rats

Catecholamine accumulation in tissues of thiamine-deficient rats after inhibition of monoamine oxidase