Study shows how mutant huntingtin protein triggers brain cell death
In 1993, scientists discovered that a single mutated gene, HTT, caused Huntington s disease, raising high hopes for a quick cure. Yet today, there s still no approved treatment.
One difficulty has been a limited understanding of how the mutant huntingtin protein sets off brain cell death, says neuroscientist Srinivasa Subramaniam, Ph.D., of Scripps Research, Florida.
In a new study published in
Nature Communications on Friday, Subramaniam s group has shown that the mutated huntingtin protein slows brain cells protein-building machines, called ribosomes.
The ribosome has to keep moving along to build the proteins, but in Huntington s disease, the ribosome is slowed. The difference maybe two, three, four-fold slower. That makes all the difference.
Huntington s Disease Found to Slow Down Ribosomes and Protein Synthesis
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Huntington s disease driven by slowed protein-building machinery in cells
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