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Motor Neuron Disease Caused By Cellular ‘Traffic Jam’

found that, in , a form of motor neuron disease causes a traffic jam of in the neurons’ outer . The scientists learned that the – cells that control muscle movement – were unable to transport cargo such as signaling molecules and proteins in need of recycling from the tips of their back to the main hub of the cell.

To study how motor neuron disease affects the body’s neurons, researchers duplicated a genetic change found in patients with an inherited motor neuron disease. The protein affected by the genetic change, p150glued, is a piece of a transporter that delivers materials from the outer reaches of the cell to its central core. Like the people with some types of motor neuron disease, the fruit flies developed progressive paralysis and died early; they also couldn’t fly. The scientists observed that in normal neurons, the cargo moved from one end of the appendages all the way back to the main hub of the cell, but in the defective neurons, the cargo at the very ends of the appendages was stuck there. The cargo along the main appendages moved normally.

“By determining what goes wrong on the cellular level in motor neuron disease, we can begin to develop therapeutics that mitigate these effects to treat the disease,” says , M.D., Ph.D., assistant professor of neurology and neuroscience at the Johns Hopkins University School of Medicine.

Johns Hopkins researchers at American Society of Cell Biology Annual Meeting

Poster: 1786/B231

Special Interest Subgroup: Dynein

Tuesday, Dec. 18, 2012; Exhibit Halls A-C, 12:30 – 2 p.m.

Authors: J. Machamer, S. Collins, Y. Yang, S. Collins and T. Lloyd


Johns Hopkins Medicine