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HOUSTON - (Feb. 21, 2012) - The Jan and Dan Duncan Neurological Research Institute (NRI) at Texas Children's Hospital, one of the world's first research institutes dedicated to basic research on childhood neurological diseases, has received a major research grant from the W. M. Keck Foundation, which supports pioneering discoveries in medical research, science and engineering. The $1 million grant will fund in vivo analysis of neural circuitry in mouse models of Rett and Angelman syndromes. These devastating childhood neurological disorders are classified as part of the autism spectrum disorders that affect about three percent of children in the U.S.
"Our researchers have been using a multidisciplinary approach studying mouse models of Rett and Angelman syndromes in the hope of understanding how the mutant genes lead to intellectual disabilities and features of autism," said Dr. Huda Zoghbi, director of the NRI at Texas Children's Hospital, professor of molecular and human genetics, neurology, neuroscience, and pediatrics at Baylor College of Medicine (BCM), and investigator, Howard Hughes Medical Institute. "The Keck Foundation grant will enable us to perform key experiments to gain insight into how these disorders alter the brain's neural network and result in behavioral abnormalities. Understanding the changes in the neural circuitry will provide a framework for investigating interventions that might prove beneficial."
Consistent with the NRI's collaborative approach, a team of researchers from different disciplines will perform different types of neurophysiological studies. As well as Dr. Zoghbi, the NRI team includes two neurosurgeons, Daniel Curry, M.D., Director of Functional Neurosurgery at Texas Children's Hospital and assistant professor in Neurosurgery at BCM, and Akash Patel, M.D., a neurosurgeon completing his residency at BCM; Daoyun Ji, Ph.D., an expert in large-scale single-unit tetrode recordings in rodents and assistant professor of Molecular and Cellular Biology at BCM; Jianrong Tang, Ph.D., an in vivo neurophysiologist and assistant professor in Pediatrics at BCM; and Yuri Dabaghian, Ph.D., a physicist and computational neuroscientist who is an assistant professor at both BCM and Rice University.
The use of freely-moving animal models will enable researchers to generate a neurophysiological map of each disease model. The researchers will then explore responses to treatments including deep brain stimulation (DBS), pharmacologic and molecular therapies.
"To our knowledge, this is the first study to investigate and compare the neural networks of two genetically-distinct models of intellectual disability and autism using freely-moving mice," said Dr. Zoghbi.
Zoghbi added that, if successful, this research funded by the W. M. Keck Foundation will help identify possible interventions for clinical features shared by a devastating class of neurological disorders.
For more information about the Jan and Dan Duncan Neurological Research Institute at Texas Children's Hospital, visit www.nri.texaschildrens.org.
About Texas Children’s Hospital
Texas Children’s Hospital, a not-for-profit health care organization, is committed to creating a healthier future for children and women throughout the global community by leading in patient care, education and research. Consistently ranked as the best children’s hospital in Texas, and among the top in the nation, Texas Children’s has garnered widespread recognition for its expertise and breakthroughs in pediatric and women’s health. The hospital includes the Jan and Dan Duncan Neurological Research Institute; the Feigin Center for pediatric research; Texas Children’s Pavilion for Women, a comprehensive obstetrics/gynecology facility focusing on high-risk births; Texas Children’s Hospital West Campus, a community hospital in suburban West Houston; and Texas Children’s Hospital The Woodlands, a second community hospital planned to open in 2017. The organization also created the nation’s first HMO for children, has the largest pediatric primary care network in the country and a global health program that’s channeling care to children and women all over the world. Texas Children’s Hospital is affiliated with Baylor College of Medicine. For more information, go to www.texaschildrens.org. Get the latest news by visiting the online newsroom and Twitter at twitter.com/texaschildrens.