Benders'dictionary of nutrition and food technology

496results in central nervous system damage, the wernicke–korsakoff syndrome.See also thiochrome; transketolase.VITAMIN B 1vitamin B 1 dependency syndromes A very small number of childrenhave been reported with a variant form of maple syrupurine disease in which the defect is in the binding of thiamindiphosphate to the branched chain keto acid dehydrogenase (EC1.2.4.4). These children respond well to supplements of largeamounts of vitamin B 1 , without the need for strict control of theirintake of the amino acids.vitamin B 2 Riboflavin. Coenzyme in a wide range of oxidationreactions of fats, carbohydrates and amino acids, as riboflavinphosphate (flavin mononucleotide), flavin adenine dinucleotideor covalently bound riboflavin at the active site of the enzyme.Riboflavin-dependent enzymes are collectively known asflavoproteins.Deficiency impairs energy-yielding metabolism and results ina group of symptoms known as ariboflavinosis, including crackingof the skin at the corners of the mouth (angular stomatitis),fissuring of the lips (cheilosis) and tongue changes (glossitis);seborrhoeic accumulations appear around the nose and eyes. Notfatal because there is very efficient recycling of riboflavin indeficiency.See also glutathione reductase; lumichrome; lumiflavin.VITAMIN B 2