Research

Fatal neurodegenerative disease in kids also affects the bowel

Researchers at WashU Medicine have described the neurodegeneration that occurs in the nervous system of the bowel in Batten disease, a rare and fatal genetic condition. In their latest study, a team led by Jonathan Cooper, professor of pediatrics (left), showed that gene therapy to the bowel in mice modeling Batten disease reduced symptoms and extended lifespan. Also pictured are co-first authors Ewa Ziółkowska (center), a postdoctoral research associate, and Matt Jansen, a graduate student. (Photo: Matt Miller/WashU Medicine)
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As a leading researcher of rare diseases that affect children’s brains, Jonathan D. Cooper, PhD, thought little about the gastrointestinal (GI) system. That is, until the parents of children with a condition that Cooper studies urged him to investigate why debilitating digestive issues troubled their kids, who suffer from an incurable and fatal neurodegenerative brain condition called Batten disease.