Weidong Wang, Ph.D.
National Institute on Aging, National Institutes of Health

Characterization of a Novel Protein Complex Involved in the Human Premature Aging Disease - Werner Syndrome.

Werner Syndrome (WS) is a rare human genetic disease with many features of premature aging. The patients usually appear normal during their teenage years. But later they prematurely develop several age-related diseases, including artherosclerosis, osteoporosis, diabetes, malignant neoplasm and cataracts. They also exhibit an aged appearance when they are still young, with features such as skin atrophy and early graying of hair. Cells obtained from WS patients also have a shortened life-span compared to those taken from normal human beings of similar age, and they are more similar to those from older people. The disease has therefore been considered by the research community as a model that can be compared to normal human aging.

Other groups have previously identified the gene defective in WS patients. However, it is unclear how it causes the age-related symptoms. Dr. Wang's group has now purified the WS gene product from normal human cells and found that it is one component of a large protein complex. They subsequently identified most of the other components within the complex, which enabled them to determine the exact cellular process in which the WS gene product functions. Their work links aging to a specific cellular process and provides a molecular mechanism for how premature aging may occur in WS patients.

Contact Dr. Wang