Rare Brain Disease Yields Clues About Broader Neural Pathology

Alexander disease, the devastating brain disease that strikes young or old, typically results from mutations in a gene known as GFAP (glial fibrillary acidic protein), leading to the formation of astrocytes. According to a report in the Journal of Neuroscience, researchers at University of Wisconsin-Madison demonstrate that Alexander disease also affects neurons, and in a way that impacts cognitive brain function. While GFAP is usually found in astrocytes, it also occurs in neural stem cells. However, in the tests conducted on mice engineered to contain the same GFAP mutation found in humans with Alexander disease, neural stem cells are present in the afflicted mice, but they fail to develop into neurons. Lab leader Albee Messing says that the study “suggest[s] that the problems in Alexander disease extend beyond just the white matter and astrocytes, and may provide a clue to the problems with learning and memory that are such prominent features in the human disease.” Moreover, the findings may provide insight into other neural diseases.  Click here to read the full article.