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Finding Shows Muscular Dystrophy-Causing Receptor Has Broader Role in Brain Development

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Finding Shows Muscular Dystrophy-Causing Receptor Has Broader Role in Brain Development

Dr. Holly Colognato and MD/PhD Student Himanshu Sharma view stem cell niche cells that are affected when brains lack the muscle cell receptor dystroglycan. 

Stony Brook, NY, September 14, 2016 – Researchers at Stony Brook University have discovered that dystroglycan, a muscle cell receptor whose dysfunction causes muscular dystrophy, actually has a critical role in brain development. The finding, published in the journal
Developmental Cell
, may help to explain why a subset of children born with a dysfunction of this muscle receptor, also have neurological problems that can include seizures, intellectual disability, autism, and severe learning disabilities.

In the newborn brain, one of the critical changes that occurs is that specialized pockets form that serve to house and nurture neural stem cells throughout life in discrete regions termed stem cell niches. Lead author Holly Colognato, PhD, Associate Professor in the Department of Pharmacological Sciences at Stony Brook University, and her team found that dystroglycan is needed to help build one of the major neural stem cell niches of the adult brain. They detailed their findings in the paper, titled “Dystroglycan suppresses Notch to regulate stem cell niche structure and function in the developing postnatal subventricular zone.”

“We found that without dystroglycan, the support cells that normally surround the stem cells in this niche fail to develop appropriately,” said Dr. Colognato. “In this “dysfunctional niche”, the stem cells overproduce oligodendroglia, a neural cell type that establishes rapid communication pathways in the brain. But overproduction of oligodendroglia can cause major abnormalities in brain wiring, thus resulting in a multitude of cognitive abnormalities,” she explained.

In light of their findings, Dr. Colognato and Mr. Himanshu Sharma, a Stony Brook MD/PhD student, are conducting laboratory experiments to test and alter dystroglycan interactions in the injured adult brain or in aging brains, where extra brain cells could be beneficial for repair or replacement.


About Stony Brook University 

Part of the State University of New York system, Stony Brook University encompasses 200 buildings on 1,450 acres. Since welcoming its first incoming class in 1957, the University has grown tremendously, now with more than 25,000 students and 2,500 faculty. Its membership in the prestigious Association of American Universities (AAU) places Stony Brook among the top 62 research institutions in North America. U.S. News & World Report ranks Stony Brook among the top 100 universities in the nation and top 40 public universities, and Kiplinger names it one of the 35 best values in public colleges. One of four University Center campuses in the SUNY system, Stony Brook co-manages Brookhaven National Laboratory, putting it in an elite group of universities that run federal research and development laboratories. A global ranking by U.S. News & World Report places Stony Brook in the top 1 percent of institutions worldwide.  It is one of only 10 universities nationwide recognized by the National Science Foundation for combining research with undergraduate education. As the largest single-site employer on Long Island, Stony Brook is a driving force of the regional economy, with an annual economic impact of $4.65 billion, generating nearly 60,000 jobs, and accounts for nearly 4 percent of all economic activity in Nassau and Suffolk counties, and roughly 7.5 percent of total jobs in Suffolk County.

Greg Filiano
Media Relations Manager, School of Medicine, Stony Brook University
Office: 631.444.9343

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