Jun Li, M.D., Ph.D., professor and chair of the Department of Neurology at the Wayne State University School of Medicine, recently received a $246,172 R-21 grant from the National Center for Advancing Translational Sciences of the National Institutes of Health to develop new strategies for assessing appropriate treatments of peripheral nerve diseases.
Charcot-Marie-Tooth disease is one of the most common inherited peripheral nerve diseases, affecting nearly 126,000 people in the United States and 2.6 million people worldwide, according to the NIH. CMT causes progressive muscle weakness, painful foot deformities and difficulty walking. As the disease progresses, weakness and muscle atrophy occur in the hands, creating difficulty with fine motor skills.
Dr. Li said the goal of the project is to advance the study of disease progression of CMT with magnetic resonance imaging biomarkers in hopes of expanding basic research to clinical trial readiness.
“Although there are promising treatments for CMT, evaluations of potential treatments are hindered by a lack of responsive biomarkers,” Dr. Li said. “Our research aims to focus on nerve MRI methods that we have developed to offer important information and improve our ability to monitor disease progression in patients through MRI-based biomarkers of CMT.”
Through the nerve MRI methods that Dr. Li and his research team have developed, they can directly image the pathology of interest within nerves themselves, which will offer complementary information and improve the ability to monitor disease progression in patients with CMT.
“Published results from our labs indicate that sciatic nerve magnetization transfer ratios (MTR), which are sensitive to myelin content changes from dysmyelination and axonal loss, relate to disability across CMT subtypes,” Dr. Li said. “Our additional preliminary data indicate that nerve MTRs are responsive to disease progression.”
The research team hopes these studies will provide them with new methods to better evaluate potential treatments for CMT, ultimately moving them to clinical trials more quickly.
Richard Dortch, Ph.D., associate professor of imaging research at the Barrow Neurological Institute at Dignity Health St. Joseph’s Hospital and Medical Center in Phoenix, is the project’s co-principal investigator.
The project number for this National Institutes of Health award is 1R21TR003312-01A1. For more information, visit https://reporter.nih.gov/search/6aKhoI0mwEycTh9IlKrkOg/project-details/10187113.