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Chediak-Higashi syndrome

Chédiak-Higashi syndrome is a rare childhood autosomal recessive disorder that affects multiple systems of the body, which arises from a mutation that causes silvery hair. It is a disease with impaired leukocyte function due to failure of phagolysosome formation – As a result of disordered intracellular trafficking there is impaired lysosome degranulation with phagosomes. In addition secretion of lytic secretory granules by cytotoxic T cells is also affected. The disease is characterised by large lysosome vesicles in phagocytes who have poor bactericidal function leading susceptibility to infections, abnormalities in nuclear structure of leukocytes, anaemia, hepatomegaly.

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John R. Gorham Distinguished Scientist of 1991 - interview - Forum - Column
From Agricultural Research, 3/1/92 by Jim Henry

"For creative research and inspiring leadership resulting in solutions to problems of disease control and basic knowledge of viral and genetic diseases of humans and animals."

But that citation barely hints at the real story behind the plaque given to Dr. Gorham for wide-ranging accomplishments over an illustrious career.

The table of organization identifies him as a veterinary medical officer at the Agricultural Research Service's Animal Disease Research Unit in Pullman, Washington, located on the campus of Washington State University. Officially, he is the eleventh annual recipient of the Distinguished Scientist Award. the top honor for scientific achievement in the U.S. Department of Agriculture.

But a look behind the scenes finds nearly 50 years of dedicated research that has improved the lives of millions of people and animals alike.

For example, his work describing the Chediak-Higashi syndrome in animals provided an important clue in the study of a devastating genetic disorder in humans. He was part of a team that first reported a viral disease of goats that causes arthritis and encephalitis. This virus disease now serves as a model for the study of arthritis and AIDS in humans.

He developed a spray vaccine for the control of distemper in mink which is used worldwide for millions of immunizations. He was also the first to discover that vitamin E prevents steatitis, a serious nutritional disease of mink, swine, and cats.

When he was a young researcher, he and a coworker discovered a new rickettsial disease of foxes and dogs that is transmitted by a small intestinal parasite.

Recently, in an interview with ARS information staffer Jim Henry, Dr. Gorham shared some personal insights into the forces that mold and motivate scientific excellence.

AR: The extensive list of your accomplishments suggests that you take a results-oriented approach toward veterinary medicine.

Gorham: Yes, it's fair to say that we're always looking for the practical application of our work. All our projects are to develop either a diagnostic test or a new vaccine--something to control disease. But in most cases it takes basic

AR: You speak of "our projects." How does your work mesh with the work of others? Where does the collaborative process begin?

Gorham: You start out by asking a question and then go about trying to solve it. And in the process of solving it, you find good colleagues. People brighter than yourself. Then you let them alone in the lab to have fun.

AR: Fun?

Gorham: If you don't have fun doing research, you might as well go out and sell insurance or real estate. Think of research as a sport, an organized sport. You sit around on a coffee break and figure out the strategy of the research. How much money you've got to spend on the project. And then you go back and attack the project.

AR: In mapping out a research project, how do you decide on a plan of attack?

Gorham: I think you have to be flexible-just like in a football game. Sometimes you go one step at a time, like old Woody Hayes' 3 yards and a cloud of dust, again and again. Sometimes in science, one small finding follows another, again and again. But then, sometimes you can throw a pass and cover a lot of ground quickly to score a touchdown. Score a new discovery, for example. Some new contribution to the team effort.

AR: But how do you remain motivated when, as happens in science as well as football, instead of gaining ground, you seem to lose it?

Gorham: Whether you seem to be ahead of the game or not, I don't think any research worker worth his or her salt is ever satisfied. You're always looking for the next finding. Something good. This is a competitive game. Something that all researchers want more than anything else is the esteem of their peers. The esteem of your contemporaries. And how do you earn the respect of your peers? You're first. And that's what it's all about. That's the fun about research. And that's what's kept me in it almost 50 years.

COPYRIGHT 1992 U.S. Government Printing Office
COPYRIGHT 2004 Gale Group

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