Why Huntington’s disease attacks brain cells in human


WASHINGTON: Recent study conducted on mice has given more insight into how and why Huntington’s disease attacks and destroys certain brain cells in human beings.

The study was published 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,” said Dr. Srinivasa Subramaniam, Department of Neuroscience at Scripps Research-Florida.

According to the study, 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. 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 three to seven people out of 100,000 have the disease and it has mostly affected those with European ancestry.

However, Subramaniam believed the disease is underreported in other areas, including India. “There is a lot of stigma associated with the disease,” said Subramaniam.

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 Huntington’s disease protein travels in the live brain. (ANI)