Publication date: May 11, 2019
JUPITER, Fla. –May 10, 2019–A toxic protein linked to Huntington’s disease can move from neuron to neuron through a nanotube tunnel whose construction is initiated by a protein called Rhes, say scientists at Scripps Research.
The finding, by Scripps Research neuroscientist Srinivasa Subramaniam, PhD, improves understanding of how and why this disease attacks and destroys certain brain cells.
“We are excited about this result because it may explain why the patient gets the disease in this area of the brain called the striatum,” says Subramaniam, an associate professor in the Department of Neuroscience at Scripps Research-Florida.
People with Huntington’s disease inherit a damaged protein that is somehow complicit in destroying brain cells.
They inserted the Huntington human disease protein into the mouse brain cells, tagged it with fluorescence and then watched as it crossed over and crawled up to enter the neighboring cell.
The Rhes protein exists in both mouse and human brains sick with Huntington’s disease.
In 2009 study, Subramaniam found that Rhes also alters the Huntington disease protein’s structure making it more toxic to brain cells.
Subramaniam’s group continues to investigate what other proteins may be helping with tunnel construction and if other disease proteins move along these membranous highways.
His laboratory is also developing ways to identify how the Huntington’s disease protein travels in the live brain.
- uniQure Announces Featured Presentations of New Data on Spinocerebellar Ataxia Type 3 at the 2019 American Academy of Neurology Annual Meeting