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A genetic model for hereditary spastic paraplegia (HSP) disease

to study how spastin functions in living neurons, the researchers designed transgenic flies that possess altered amounts of spastin protein in their neurons and assessed the effects. Using this approach, they found that spastin protein localizes to synapses, specialized sites of neuronal communication, where it acts to locally destabilize microtubules. Moreover, the researchers found that specific drugs that alter microtubule stability appear to remedy defects that occur in synaptic function as a result of changes in neuronal spastin levels. All told, the study provides intriguing new insight into how a genetic mutation that alters microtubule function may disrupt the way neurons communicate and offers a new line of thinking about possible treatments for the debilitating HSP condition.


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Contact: Heidi Hardman
hhardman@cell.com
617-397-2879
Cell Press
12-Jul-2004


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