Trouble in the cell's powerhouse

摘要

In Greek, 'ataxic' (aτraξc,) means 'disordered'. In neurological diseases known as ataxias, the lack of order is mainly one of movement: patients cannot coordinate voluntary movements because of the loss of certain neural circuits, making it difficult for them to walk, reach for objects and speak. Increasingly, researchers are finding that the underlying cause of some hereditary ataxias is another sort of disorder - that of mitochondria, the intra-cellular organelles that act as the cell's powerhouses. However, we know very little about the mechanisms that trigger certain other hereditary ataxias, such as the condition known as autosomal recessive spastic ataxia of Char-levoix-Saguenay, or ARSACS.