(NEUROIMAGINGANDNEUROCIRCUITRY(NNC):COREE) PROJECT SUMMARY Description: The Neuroimaging and Neurocircuitry Core (NNC) enables the study of changes in brain circuitry which occur in people with IDD. The Core supports research in both patients and animal models by providing users the following state-of-the-art technologies: (a) Magnetoencephalographic (MEG) recordings of brain activity; (b) Magnetic resonance imaging (MRI) studies of brain structure and function; (c) Multiphoton, confocal, and epifluorescence microscopic imaging studies of the dynamics of neuronal circuit function; (d) Patch clamp recording studies in neurons; and (e) Electroencephalographic (EEG) and other in vivo recordings. Analyses are performed in affected human patients with IDD, in Preclinical animal models of IDD, and in vitro (stem cells and tissue culture). Core Directors provide users with access to a wide set of otherwise inaccessible neuronal circuit assessment techniques, as well as expert consultation regarding experimental design and analysis. In all core functions, emphasis is placed upon quality control. In addition to assuring that all studies are competently conducted and appropriately analyzed, the Core Directors assist users to design studies that are properly controlled, adequately powered, and, when feasible, conducted in a blinded fashion. Relevance to IDDRC Mission: The overarching theme of this IDDRC is ?Genes, Brain & Behavior? (see Overview). This Core focuses on each of these overlapping domains. Genetic alterations underlie many IDDs, and result in aberrant protein expression, which alters basic brain function. This in turn generates the behavioral anomalies which constitute IDD. The study of patients and animal models brings the genetic components into sharp focus, and the NNC facilitates examination into how these genetic changes alter brain function and, subsequently, whether such changes are correlated to behavior. Thus, the goal of the Core is to quantify the changes in function of neuronal circuits which result from genetic mutation and variation. Elucidating the changes in dynamic function of neuronal circuitry responsible for the generation of the pathologic behaviors collectively termed the IDD is critical in translational efforts to develop better therapies to treat and cure brain pathologies underlying the IDDs and other related CNS disorders such as autism, epilepsy, and cerebral palsy. Eligibility: These services are available both to approved users of the IDDRC at CHOP/UPenn and to users at other Centers in the Network.

National Institute of Health (NIH)
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Specialized Center--Cooperative Agreements (U54)
Project #
Application #
Study Section
Special Emphasis Panel (ZHD1)
Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
Children's Hospital of Philadelphia
United States
Zip Code
Niarchou, Maria; Calkins, Monica E; Moore, Tyler M et al. (2018) Attention Deficit Hyperactivity Disorder Symptoms and Psychosis in 22q11.2 Deletion Syndrome. Schizophr Bull 44:824-833
Sun, Daqiang; Ching, Christopher R K; Lin, Amy et al. (2018) Large-scale mapping of cortical alterations in 22q11.2 deletion syndrome: Convergence with idiopathic psychosis and effects of deletion size. Mol Psychiatry :
Ugras, Scott; Daniels, Malcolm J; Fazelinia, Hossein et al. (2018) Induction of the Immunoproteasome Subunit Lmp7 Links Proteostasis and Immunity in ?-Synuclein Aggregation Disorders. EBioMedicine 31:307-319
Polyak, Erzsebet; Ostrovsky, Julian; Peng, Min et al. (2018) N-acetylcysteine and vitamin E rescue animal longevity and cellular oxidative stress in pre-clinical models of mitochondrial complex I disease. Mol Genet Metab 123:449-462
Grissom, N M; McKee, S E; Schoch, H et al. (2018) Male-specific deficits in natural reward learning in a mouse model of neurodevelopmental disorders. Mol Psychiatry 23:544-555
He, Zhuohao; Guo, Jing L; McBride, Jennifer D et al. (2018) Amyloid-? plaques enhance Alzheimer's brain tau-seeded pathologies by facilitating neuritic plaque tau aggregation. Nat Med 24:29-38
Estes, Annette; Munson, Jeffrey; John, Tanya St et al. (2018) Parent Support of Preschool Peer Relationships in Younger Siblings of Children with Autism Spectrum Disorder. J Autism Dev Disord 48:1122-1132
Young, Jami F; Jones, Jason D; Sbrilli, Marissa D et al. (2018) Long-Term Effects from a School-Based Trial Comparing Interpersonal Psychotherapy-Adolescent Skills Training to Group Counseling. J Clin Child Adolesc Psychol :1-9
Barca, Emanuele; Ganetzky, Rebecca D; Potluri, Prasanth et al. (2018) USMG5 Ashkenazi Jewish founder mutation impairs mitochondrial complex V dimerization and ATP synthesis. Hum Mol Genet 27:3305-3312
Pallathra, Ashley A; Calkins, Monica E; Parish-Morris, Julia et al. (2018) Defining behavioral components of social functioning in adults with autism spectrum disorder as targets for treatment. Autism Res 11:488-502

Showing the most recent 10 out of 223 publications