Advancing knowledge of human development, developmental disabilities, and neurodegenerative diseases.
Alexander disease (AxD) is a primary genetic disorder of astrocytes caused by dominant mutations in the gene encoding the intermediate filament (IF) protein GFAP. This disease is characterized by excessive accumulation of GFAP, known as Rosenthal fibers, within astrocytes.
Researchers at the University of Wisconsin–Madison have made a surprising and potentially crucial discovery about Alexander disease, a rare and fatal neurological disorder with no known cure. Using a mouse model for this disease, which in humans involves the destruction of white matter in the brain, a research team led by Albee Messing, director of …
May 18, 2016 Adityarup “Rup” Chakravorty, Waisman Communications Jelte Rijkaart is 10 years old, with a ready smile, dark brown hair and warm brown eyes. He enjoys hanging out with people, especially his brother Roan. He is passionate about clocks—he has a wall dedicated to cuckoo clocks—and enjoys all sorts of movies about clocks on …
TDP-43 mislocalization in a mouse model of Alexander disease Legend: Mislocalized TDP-43 colocalizes with GFAP in GfapR236H/+ mice. TDP-43 (red) and GFAP (green) immunolabeling in coronal brain sections from 10-week-old mice. Nuclei are labeled with DAPI (blue). In WT mice, TDP-43 is primarily localized to neuronal nuclei (pyramidal layer of the hippocampus shown here), with very little colocalization with GFAP. In GfapR236H/+mice, TDP-43 …
A fruit fly model of a rare, neurodegenerative disease is helping researchers trace the series of steps that lead to neuronal cell death. Damage to astrocytes - star-shaped cells found in the brain and spinal cord - is found in many neurodegenerative conditions, but it's been unclear exactly what role astrocyte dysfunction plays in the development of disease.
Alexander disease is a devastating brain disease that almost nobody has heard of — unless someone in the family is afflicted with it. Alexander disease strikes young or old, and in children destroys white matter in the front of the brain.
The telegram from President John F. Kennedy to University of Wisconsin President Fred Harrington was both eerie and visionary. Eerie because it was delivered Nov. 20, 1963 – just two days before Kennedy was assassinated in Dallas – and visionary because it seemed to anticipate the challenges confronting science in its quest to explore the human brain.
Using novel screens to sort through libraries of drugs already approved for use in human patients, a team of Wisconsin researchers has identified several compounds that could be used to treat a rare and deadly neurological disorder.
"Don’t give up, my love, or I’ll give up with you, because I only live to see the fulfillment of this dream: that you may continue to live. Yours is a life sentence, not a death sentence.”
