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Prof`s work with Salk Institute is personal

2.5 from 4 votes
Sunday, June 01, 2008

Zitzer-Comfort brings a personal dimension to her research through her 19-year-old daughter Heidi, who was diagnosed with Williams Syndrome when she was 4. The goal of Zitzer-Comfort`s study was to compare the social behavior of children with Williams Syndrome - known for their innate drive to interact with people - across cultures with different social mores.

Using a questionnaire developed by Salk researchers, Zitzer-Comfort and lead author Ursula Bellugi, director of the Laboratory for Cognitive Neuroscience at the Salk Institute, asked parents in the United States and Japan to rate the tendency of their children to approach others, their eagerness to please, their tendency to empathize with others` feelings and the tendency for other people to approach their children. Despite the differences in upbringing, in both countries children with Williams Syndrome were rated significantly higher in global sociability and their tendency to approach strangers than were their more typical counterparts. But cultural expectations clearly influence social behavior, since the sociability of typically developing American children was on par with Japanese children with the syndrome, whose social behavior is considered out of bounds in their native country.

"It really is an intriguing illustration of the interaction between nature and nurture," said Zitzer-Comfort, who joined the university in 2005. But she notes that there might be alternative explanations. Japanese parents rated their children generally lower on the questionnaire`s seven-point scale. "Perhaps the stigma of having a `different` child in Japan affected the ways in which parents ranked their child`s degree of sociability," she said.

Identified more than 40 years ago, Williams Syndrome occurs in an estimated one in 20,000 births worldwide. It arises from a faulty recombination event during the development of sperm or egg cells. As a result, almost invariably the same set of about 20 genes surrounding the gene for elastin is deleted from one copy of chromosome seven. Despite health problems and a generally low IQ, individuals with Williams Syndrome are loquacious and sociable.

When her daughter was diagnosed in 1991, Zitzer-Comfort used the research skills she honed in her study of English Education and Native American literature to learn all she could about Williams Syndrome.

"There was little research and not much was known but I went out and read everything I could find," she recalled. "Then I visited the Salk Institute. They`ve been doing pioneering research for years. Williams Syndrome was first described as a unique entity in 1963 but it is only in the last 12 years that scientists have figured out exactly which genes are involved."

With all the emphasis on genetics today, it is important to remember the impact of culture and family on personality. "We are more than the sum total of our genes," she said. "We represent a dynamic interaction between our genes and our environment. The same holds true for those with genetic disorders as much as it does for the rest of us. That is especially important for families because they can have the same impact on those with disabilities as they can on anyone else."

She praises the Salk Institute for its research leadership. "I am impressed by their tireless efforts to raise awareness of Williams Syndrome," she said. "The awareness has grown into a phenomenon. It bridges research into the brain, human behavior, genetics and cognition. Geneticists are interested in the syndrome because they know exactly what genes are involved and they are beginning to tie those genes into brain and behavior. It has gone from being something that no one heard about and misdiagnosed as mental retardation to something more."

Copyright California State University

Author: By Richard Manly
Source: California State University, Long Beach
2.5 from 4 votes
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