Other scientists have discovered that an enzyme, transglutaminase, usually involved with skin formation and healing, appears to be especially active in the brains of people with Huntington's disease. The discovery was made by studying brains during autopsy. They theorize it could be the "smoking gun" behind formation of the disease and therefore the key to the cure.

That discovery was made by a team of U.S. and Israeli scientists. It backs up a theory put forth in 1993 by a Harvard professor, Howard Green, that the enzyme might act as a catalyst for gathering together a protein called huntingtin. Huntingtin is found in clumps in the brain cells of people with Huntington's disease. It's not found elsewhere in their bodies.

A pharmaceutical company, Regeneron Pharmaceuticals, and Medtronic Inc. have also entered into a joint development agreement to collaborate on research and development of a family of treatments for central nervous system diseases. They'll use the pharmaceutical company's experimental treatments and the Medtronic delivery systems. They're especially hopeful that an implantable pump will be able to infuse a medication into the brains of people with Huntington's disease.

The May 20 issue of Nature said scientists have learned that the enzyme caspase-1, present in the brains of both humans and mice, could be important to development of Huntington's disease. By inhibiting the enzyme in the brains of mice that have a syndrome with characteristics of Huntington's, they were able to delay the disease's progression. Caspase-1 is believed to help trigger the death of brain cells. When the enzyme was inhibited, the mouse's life was extended by 20 percent compared to those without the inhibition. And the disease was significantly delayed.

They're hoping that further tests will show it works in human brains, too. But they're nowhere near ready to go for human trials yet. And treatment based on the research, even if it's successful, is years away.

That's all wonderful news, says Robert Bishop. Things are beginning — finally — to happen. But the budget for Huntington's research is miniscule compared to other diseases. It has long been, and remains, at the bottom of the priority list. The fact that it's gaining any attention thrills him.

But it has come, without question, too late to help Amy or Julie or John.

Now the Askews and the Bishops can only hope it's not too late to change Danny's future. And that of his nieces and nephews.

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