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Comprehensive Long-Term Evaluation of the Gunn Rat Kernicterus Model

Principal Investigator:
S. Ali

The proposed project is the initial phase of a long-term endeavor to develop therapies that will help patients with dyskinetic cerebral palsy related to mitochondrial dysfunction and a Kernicterus-like phenotype. We have evidence that the combination of a genetic predisposition affecting mitochondrial function will reduce the threshold for perinatal brain injury and can result in a distinct clinical picture in children born prematurely. Several patients have been identified at the Kennedy Krieger Institute with a very similar clinical phenotype.

All are infants born extremely premature, who did not suffer from significant hyperbilirubinemia (jaundice) but developed a "Kernicterus-like" picture with sensory-neural hearing loss and globus pallidus lesions leading to severe dystonia. We have identified mitochondrial dysfunction in these patients as assessed by metabolic laboratory studies and/or mtDNA analysis.

While a rat model for Kernicterus has been reported, presenting with dystonia, sensory-neural hearing loss and cerebellar hypoplasia, no long-term neurobehavioral or neuroimaging studies have been reported. Furthermore, while it is known that the increased bilirubin is a potent mitochondrial toxin, the degree of mitochondrial dysfunction in this model has not been studied.

Bradley L. Schlaggar, M.D., Ph.D., Named President and CEO of Kennedy Krieger Institute

We’re thrilled to welcome Bradley L. Schlaggar, M.D., Ph.D., to the Kennedy Krieger family as our next President and CEO.
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