Classic galactosemia and generalised epimerase deficiency galactosemia are caused by defects in key enzymes, GALT and GALE, respectively, responsible for the metabolism of dietary galactose. Both disorders can have severe effects, in extreme cases leading to neonatal death, and their prognosis and treatment remain problematic, partly due to lack of a good animal model. Judith Fridovich-Keil and colleagues present data in two linked papers describing Drosophila models for loss of GALT and GALE, and demonstrate that important aspects of the human disease phenotypes are also found in flies.
2010