PUBLIC RELEASE DATE:

19-Nov-2014
In a serendipitous finding, Johns Hopkins neuroscientist Bindu Paul, Ph.D., connected the manufacture of an essential amino acid to Huntington’s disease, changing scientific understanding of the condition. Studying mice lacking the enzyme that makes cysteine, Paul, a researcher in the laboratory of Solomon Snyder, M.D., noticed the mice behaved like those used to study Huntington’s disease: They remained still and clasped their paws together when dangled by their tails. Intrigued, Paul checked the amount of the cysteine-making enzyme in the Huntington’s mice and found decreased levels in the disease-affected tissues. More experiments soon revealed that the mutant huntingtin protein, which causes the disease, gloms up the genetic machinery that generates the cysteine-making enzyme. Without the enzyme, much less cysteine is made. Paul fed the Huntington’s mice diets rich in cysteine, and they regained normal behavior, swinging and biting when dangled by their tails. These unexpected findings have led to clinical trials to see if treatment with cysteine can relieve symptoms in people with Huntington’s.

Poster S9 798.08
Wed., 4 p.m., Walter E. Washington Convention Center, Halls A-C
Mei Han, Rebeca Mejias-Estevez, Richard Huganir, Tao Wang