Wilsons disease is a rare condition characterized by a build-up of copper in the liver and brain. High copper levels degrade neurological function and cause speech deficits. Unless treated, this disease is usually fatal by the late teens. Only about 5,000 persons in the United States have Wilsons disease, an orphan disease, and drug companies do not find it profitable to develop new therapies for it.
George J. Brewer, professor of human genetics, has found that the administration of zinc compounds inhibits copper retention and has pioneered this therapy for maintenance of normal copper levels. A New Drug Application to the FDA is under preparation to facilitate national licensing of zinc therapy.
Brewer has also studied a molybdenum-based compound, tetrathiomolybdate (TM), for treatment of patients acutely ill from high copper levels. The drug penicillamine has been used, but it is often not effective. Over the next three years, Brewer will conduct a double-blind study comparing TM and penicillamine therapies.
The research on TM is supported by a three-year, $430,000 grant from the Food and Drug Administration. The FDA is also supporting a three-year, $455,000 study of zinc therapy.
New technology permits biomedical science and health care researchers to gather ever more data about the human organism. While this is a boon, it also forces researchers and physicians to develop methods to organize and gain ready access to the huge amounts of data generated, much of which is visually oriented. Computerized imaging systems can create megabytes of pictures, but the growing databanks of such images are only useful if those who need them can retrieve them in a timely fashion.
Walter B. Panko, assistant dean for medical administration and professor of information and library studies, is heading a project at the Medical Center to organize, classify, retrieve and transmit data generated by imaging technologies. In this case, the development work is being done using data generated by confocal microscopy at the Digital Microscopy and Scientific Visualization facility in the Department of Anatomy and Cell Biology. The goal is to establish a national repository for confocal images that is readily accessible anywhere in the country and from any type of computer, facilitating research, educational, and clinical activities.
Co-investigators in the cross-disciplinary group for this project are Brian Athey, research fellow; Miranda Pao, professor of information and library studies; and Amy Warner, assistant professor of information and library studies. This research is supported by a three-year, $433,707 grant from the National Library of Medicine of the National Institutes of Health.
Traditionally, writing assignments have provided students with purposes, topics, and audiences that may have little relevance to their lives, and which often fail to engage the interest of African American adolescents at distressed urban high schools. As an alternative, Pamela Moss, assistant professor of education, is working with fellow researchers from the
U-M and English teachers from Henry Ford High School to develop a portfolio assessment program that encourages students to find real purposes for their writing.
Such a program asks students to draw on available resources in their school, homes, and community as they develop and share their own ideas. Portfolio assessment project classrooms promote critical, collaborative, and reflective thought while valuing the experiences of the students involved. Mosss research group is working to develop assessment strategies conducive to these environments. This project is sponsored by a two-year, $50,000 grant from the Metropolitan Life Foundation and a $51,046 U-M grant.