The finding, by Scripps Research neuroscientist Srinivasa Subramaniam, PhD, improves understanding of how and why this disease attacks and destroys certain brain cells. The research was published Friday, May 10 in the Journal of Cell Biology.
"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 - Huntington - Disease - Protein - Brain
People with Huntington's disease inherit a damaged protein that is somehow complicit in destroying brain cells. Scientists discovered this protein in 1993 but are still piecing together its role in this degenerative disease. Scans show Huntington's disease brains are shrunken and degraded. As the neurons deteriorate, people lose motor control, they can have emotional problems and their thinking and memory suffer. Symptoms usually begin around age 30 to 40 and last 15 to 20 years until death. A rarer and more aggressive form of the disease affects children, cutting their childhood and lives short.
About 3 to 7 people out of 100,000 have the disease and it has mostly affected those with European ancestry. However, Subramaniam believes the disease is underreported in other areas, including India.
Lot - Stigma - Disease - Subramaniam
"There is a lot of stigma associated with the disease," says Subramaniam.
His laboratory investigates the molecular mechanics of Huntington's disease and other neurodegenerative illnesses, including Alzheimer's and Parkinson's disease, to find potential therapy targets.
Case - Huntington - Question - Transport - Benefit
"In the case of Huntington's, the question is can we block this transport, and does it have any benefit or effect?" says Subramaniam.
For this study, Subramaniam and colleague Manish Sharma, PhD, looked at mouse neurons under a confocal microscope and saw that the cells formed sticky, string-like protrusions around 150 microns long which floated above the cells, connecting them.
Rhes - Tubes - Cells
"When I saw Rhes making these tunnel-like tubes between the cells I was...
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