Urinary excretion of pentose phosphate pathway-associated polyols in early postnatal life.

Background: Two new inborn errors in the pentose phosphate pathway have been described: ribose-5-isomerase deficiency and transaldolase deficiency. These defects are characterized by accumulation of specific polyols in body fluids. Little is known about human polyol metabolism, but there are indications for a physiological role primarily during early development.

Influence of D-arabitol and ribitol on neuronal network activity.

To date, two new defects in the pentose phosphate pathway have been identified in patients with abnormalities in their polyol profiles. Some of them presented with neurological symptoms of so far unknown aetiology. The pathophysiological role of polyols, e.