Protein fights nerve loss

A new research development holds promise that some day, people with Parkinson’s disease may be able to live free of its debilitating effects with help from a chaperone.

The chaperones in question are a class of proteins that help assemble and refold other proteins. Penn researchers have focused on a protein known as Hsp70, which, when introduced into fruit flies, prevents neurons from being destroyed by deformed proteins. New research by Professor of Biology Nancy Bonini, an investigator with the Howard Hughes Medical Institute, suggests that Hsp70 may also halt the progress of Parkinson’s, a condition often characterized by uncontrolled trembling.

In Parkinson’s, a malformed protein called alpha-synuclein destroys neurons that make dopamine, which is necessary to transmit signals to and from the brain.

Penn researchers had already used fruit flies to identify that malformed protein and to show that Hsp70 reduces the damage it causes. Then they examined the tissue of Parkinson’s disease patients and found the same deformed protein.

“We thought if [Hsp70] could suppress [other neurodegenerative diseases], it could suppress Parkinson’s,” Bonini said.

The research, which was published on the Science Express Web site Dec. 20, “gives us hope that the same mechanisms are happening in humans as in fruit flies, and that gives us hope that if you can upregulate [increase] the amount of chaperones, you can protect against neurological damage in humans the same as in flies.”

Before these results can be translated into an effective treatment for Parkinson’s disease, Bonini said, two things need to be done. Research on mammals needs to be conducted to confirm that the same process is at work in both fruit flies and mammals, and drugs need to be developed that can increase the amount of Hsp70 in the nervous system. The first step is already under way, with Professor of Pathology and Laboratory Medicine John Trojanowski and Center for Neurodegenerative Disease Research Co-Director Sun Yong Lee conducting studies on mice.

“We can also try drug studies on fruit flies and see what happens,” Bonini said.

Originally published on January 24, 2002