Tuesday, September 6, 2011

After observing his grandfather’s inability to walk unassisted due to a muscle disease, Yuji Hara had more questions than answers.

“I wanted to know why his disease was caused and how his disease could be cured,” Hara said. That was all the inspiration he needed to pursue a research career in the field of muscular dystrophy.

After completing his undergraduate and graduate studies in Japan, Hara took the advice of his mentor Dr. Yasuo Mori and accepted a postdoctoral fellowship in the Department of Molecular Physiology and Biophysics at the University of Iowa in 2005. Hara had never been to the United States before arriving in Iowa City.

Hara works with Kevin Campbell, Ph.D., professor and head of molecular physiology and biophysics at the UI Carver College of Medicine and a Howard Hughes Medical Institute investigator. Campbell came highly recommended by Mori, as the two scientists had previously collaborated on research projects.

Campbell, Hara and their colleagues published a study in the March 10 issue of the New England Journal of Medicine that impacted Hara both personally and professionally. By studying a single patient with mild muscle disease and severe cognitive impairment, Campbell‘s team identified a new mutation in the dystroglycan gene that causes a type of muscular dystrophy.

By engineering the human gene mutation into a mouse, the researchers also created a new mouse model that could help screen potential drugs to treat this type of muscular dystrophy.

This study ties together almost two decades of research on dystroglycan, an important muscle protein that is abnormal in a group of congenital muscular dystrophies, which often involve brain abnormalities.

“This paper is very important for my future career. I would like to thank Dr. Campbell and our collaborators for giving me the opportunity to contribute to this important project,” said Hara, 36, a native of Nagano, Japan. “Our data provides direct genetic evidence that dystroglycan is the main, and most likely the only, molecule that bears sugar chains whose abnormalities cause muscular dystrophy involving brain abnormalities.

“Our findings are very important for the clinical aspect, as well as for the basic research area.”

Hara played a major role in the creation of the new mouse model.

“Yuji was the major driving force for this very exciting project. He was the lead scientist both at the bench and writing the manuscript,” Campbell said.

Campbell, considered one of the leading scientists in the muscular dystrophy field, provided Hara invaluable guidance during this important project.

“(Campbell) is a great mentor, and I have learned many things from him,” Hara said. “He taught me about the attitudes and passions that are necessary for scientists. We submitted the initial manuscript to a journal in 2008, but it took two years for our manuscript to be accepted. He encouraged me during that time, and told me the importance of the research project.”

Hara has enjoyed working in Campbell’s lab for the last five years and has found Iowa City to be a safe, quiet community that allows him to focus on his research.

Later this year, Hara intends to return to Japan and hopefully begin a career in academia.