Scientists working to find treatments for a severe form of diabetes called Wolfram syndrome have identified a gatekeeper in cells that prevents harmful molecules from spilling into places where they don’t belong and triggering cell death. The researchers, at Washington University School of Medicine, also found that the gatekeeper may be a good treatment target for other disorders caused by cellular stress.
A research team led by Washington University endocrinologist Fumihiko Urano, MD, PhD, (right) and first author Simin Lu, PhD student, (left) has discovered that a commonly prescribed muscle relaxant may be an effective treatment for Wolfram syndrome, a rare but devastating form of diabetes.
The brain uses more glucose than just about any other organ in the body, and Tamara Hershey, PhD, uses brain-imaging tools to study the effects of diabetes. She also studies Parkinson’s disease, obesity, Tourette syndrome and Wolfram syndrome, learning about how fluctuations in glucose levels can influence brain function.
Fumihiko Urano, MD, PhD, has been named the new Samuel E. Schechter Professor of Medicine at Washington University School of Medicine in St. Louis. Shown (from left) are Larry J. Shapiro, MD; Urano; and Victoria J. Fraser, MD.
School of Medicine researchers have received a five-year, $2.7 million grant to detect and analyze differences in the brains of children with a rare illness, Wolfram syndrome. The disorder includes a severe form of diabetes, hearing and vision loss and kidney problems. Patients also eventually lose muscle control and coordination from brain degeneration.
The rare disorder Wolfram syndrome is caused by mutations in a single gene, but its effects on the body are far reaching. Now, researchers report that they have identified a mechanism that affects both insulin-secreting cells and neurons. The finding will aid in the understanding of Wolfram syndrome and also may be important in the treatment of milder forms of diabetes and other disorders.
Inflammation and cell stress are major factors in diabetes. Cell stress also plays a role in Wolfram syndrome, a rare, genetic disorder that afflicts children with many symptoms, including juvenile-onset diabetes. Now scientists at Washington University School of Medicine in St. Louis and elsewhere have identified a molecule that’s key to the cell stress-modulated inflammation that causes insulin-secreting cells to die.
Children with a rare syndrome that includes a form of insulin-dependent diabetes have brain abnormalities that appear to set the stage for cognitive problems later in life, according to new research at Washington University School of Medicine in St. Louis. Scientists, including Tamara Hershey, PhD, had assumed those brain changes occurred late in the disease process, but the new findings suggest that some changes occur early in childhood.
M. Alan Permutt, MD, professor of medicine and of cell biology and physiology at Washington University School of Medicine in St. Louis, died of cancer Sunday, June 10, 2012, in St. Louis. He was 72.
Ten patients ranging in age from 7-23 came to the School of Medicine in August for testing and evaluation at the first-ever multidisciplinary clinic for Wolfram syndrome.