Vitamin A could keep Hirschsprung's at bay Available to Purchase

Hirschsprung disease (HSCR) is a life-threatening developmental defect of the enteric nervous system (the ENS, which controls intestinal function) that is generally believed to be caused by genetic mutations. But now, Ming Fu, Robert Heuckeroth and co-workers reveal that vitamin A deficiency might also be a causative factor (see p. 631). Vitamin A is a precursor of retinoic acid (RA), and the authors show that mice in which RA is depleted when dietary vitamin A is removed develop HSCR-like defects, which are characterised by ENS precursors failing to migrate into the distal bowel. In mice carrying a Ret gene mutation, the most common identified cause of HSCR in humans, even mild vitamin A deficiency increased the risk of HSCR-like disease. The authors provide a cellular mechanism for these findings by demonstrating that RA is required for ENS precursor polarisation and migration. These data suggest that some cases of HSCR...