In a major medical advance, scientists have discovered what causes brain cells to die in people with Huntington’s disease and six related disorders.
The scientists said that in each case an insoluble ball of protein formed in the cell nucleus and killed it. Until now, the cause of cell death in these diseases had not been known.
Huntington’s disease is a mysterious, inherited malady in which portions of the brain, known as basal ganglia, atrophy and die. Victims develop an abnormal gait, as if being drunk, and suffer severe dementia. The related diseases, which are also inherited, include spinocellebellar ataxia and spinal and bulbar muscular atrophy. They affect different areas of the brain but produce similar symptoms.
The new findings, by researchers in Britain, Germany and the United States, are described in two articles that appear today in the journal Cell and a third article in the August issue of the journal Neuron. Researchers said they hoped to learn how to dissolve the balls of protein, thereby delaying or preventing the onset of the disease.