Experiments in lab dishes could explain why only some neurons in the brain are vulnerable to the disease
Researchers may have discovered how a neuron-killing protein selects its victims — it has an accomplice.
Scientists identified a mutant form of the protein huntingtin as the culprit in Huntington’s disease in 1993. The protein is found in every cell in the body, but it only turns deadly in brain cells — particularly cells in the striatum, a part of the brain that helps control movement. Why mutant huntingtin preferentially kills those cells has been a mystery.
Now, researchers at Johns Hopkins University in Baltimore report in the June 5 Science that a protein called Rhes may goad huntingtin into killing brain cells in the striatum, leading to Huntington’s disease. If confirmed, the finding could provide new avenues for developing therapies to treat the fatal neurodegenerative disease, says Nancy Wexler, president of the Hereditary Disease Foundation and a Huntington’s disease researcher at Columbia University.
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