Polyglutamines stop traffic: axonal transport as a common target in neurodegenerative diseases.
In this issue of Neuron, two papers provide evidence that polyglutamine (polyQ) proteins disrupt axonal transport. Gunawardena et al. show that normal levels of huntingtin are required for proper axonal transport in Drosophila. In addition, expression of expanded polyQ proteins disrupts axonal transport in larval neurons. Szebenyi and colleagues find that polyQ proteins directly inhibit fast axonal transport using axoplasm from the squid giant axon and suggest that axonal transport defects may be a common feature of polyQ disease pathogenesis.
Feany
Mel B
MB
Department of Pathology, Division of Neuropathology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115, USA.
La Spada
Albert R
AR
D000818:Animals; D006801:Humans; D001370:Axonal Transport / Q000502:physiology; D019636:Neurodegenerative Diseases / Q000378:metabolism; D010455:Peptides / Q000378:metabolism; D021381:Protein Transport / Q000502:physiology
D010455:Peptides; C097188:polyglutamine