This Center grant requests funding for seven Core facilities to support a broadly-based research program in the study of mental retardation and developmental disabilities. The seven Core facilities are: Administrative, Multimedia, Image Graphics/Confocal, Cellular Neuroscience, Gene Manipulation, Molecular Genetics, and Cell Sorter. The Cores, which are derived from the previous 10 Core facilities of the Center, have undergone major development and support the research of 49 investigators who receive approximately $14,700,000 in external funding, approximately $13,000,000 of which is from the NIH. The research is in two programmatic areas, i.e., Genetics and Neuroscience. In Genetics, directed by Dr. Louis Kunkel, there are five major research efforts: molecular genetics of neuromuscular disease, genetic imprinting in Angelman and Prader-Willi syndromes, molecular diagnosis, dystrophin-related proteins in brain, and somatic cell genetics. In Neuroscience, there are three major programs, i.e., Basic Neuroscience, directed by the newly- recruited Associate Director of the Center, Dr. Michael Greenberg; Clinical Neuroscience/Behavior, directed by the Center Director; and Developmental Biology, directed by Dr. Merton Bernfield. The Basic Neuroscience Program consists of 23 funded investigators whose research efforts span a broad spectrum of interdigitated research in molecular, cellular, ultrastructural, and systems neuroscience, with a strong overall emphasis on development. A research project in Basic Neuroscience, proposed in this grant as a """"""""New Program"""""""", addresses """"""""Mechanisms of Glutamate- Mediated Immediate Early Gene Expression"""""""" (Stephen Finkbeiner, M.D., Ph.D). A second major program in Neuroscience, i.e., Clinical Neuroscience/Behavior, directed by the Center Director, has undergone marked development in terms of investigators and scope of research. The research areas include: ischemic neonatal brain injury, neurocardiology, behavioral and neurophysiological development, developmental psychology and learning disturbances, development of human visual function, HIV and the developing nervous system, and effects of endogenous and exogenous toxins on brain development. These areas of clinical research interdigitate with the basic research in the MRRC. A third exciting program in Neuroscience, i.e., Developmental Biology, directed by Dr. Merton Bernfield, addresses fundamental mechanisms of cellular organization during embryonic development, particularly the interaction of cell surface molecules with the extracellular matrix. The multidisciplinary approaches to the research of this MRRC include the various basic science disciplines within Genetics and Neuroscience, and the clinical science fields of Neurology, Pediatrics, Neonatology, Infectious Disease, Endocrinology, Metabolism, Genetics, Cardiology, Neurosurgery, Neuropathology, Ophthalmology, Psychiatry, and Psychology. The basic science programs are housed in over 50,000 sq. ft. of the Enders Pediatric Research Building. The clinical research programs are centered in the adjacent Children's Hospital.

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Center Core Grants (P30)
Project #
5P30HD018655-17
Application #
2673497
Study Section
Mental Retardation Research Committee (HDMR)
Project Start
1996-08-01
Project End
2001-07-31
Budget Start
1998-08-01
Budget End
1999-07-31
Support Year
17
Fiscal Year
1998
Total Cost
Indirect Cost
Name
Children's Hospital Boston
Department
Type
DUNS #
076593722
City
Boston
State
MA
Country
United States
Zip Code
02115
Chen, Bo; Li, Yi; Yu, Bin et al. (2018) Reactivation of Dormant Relay Pathways in Injured Spinal Cord by KCC2 Manipulations. Cell 174:521-535.e13
Ci, Yanpeng; Li, Xiaoning; Chen, Maorong et al. (2018) SCF?-TRCP E3 ubiquitin ligase targets the tumor suppressor ZNRF3 for ubiquitination and degradation. Protein Cell 9:879-889
Sieker, Jakob T; Proffen, Benedikt L; Waller, Kimberly A et al. (2018) Transcriptional profiling of articular cartilage in a porcine model of early post-traumatic osteoarthritis. J Orthop Res 36:318-329
Leviton, Alan; Allred, Elizabeth N; Joseph, Robert M et al. (2018) Behavioural dysfunctions of 10-year-old children born extremely preterm associated with corticotropin-releasing hormone expression in the placenta. Acta Paediatr 107:1932-1936
Sieberg, Christine B; Taras, Caitlin; Gomaa, Aya et al. (2018) Neuropathic pain drives anxiety behavior in mice, results consistent with anxiety levels in diabetic neuropathy patients. Pain Rep 3:e651
Markowitz, Jeffrey E; Gillis, Winthrop F; Beron, Celia C et al. (2018) The Striatum Organizes 3D Behavior via Moment-to-Moment Action Selection. Cell 174:44-58.e17
Masuyer, Geoffrey; Zhang, Sicai; Barkho, Sulyman et al. (2018) Structural characterisation of the catalytic domain of botulinum neurotoxin X - high activity and unique substrate specificity. Sci Rep 8:4518
Cakir, Bertan; Liegl, Raffael; Hellgren, Gunnel et al. (2018) Thrombocytopenia is associated with severe retinopathy of prematurity. JCI Insight 3:
Moore, Anna R; Richards, Sarah E; Kenny, Katelyn et al. (2018) Rem2 stabilizes intrinsic excitability and spontaneous firing in visual circuits. Elife 7:
Winawer, Melodie R; Griffin, Nicole G; Samanamud, Jorge et al. (2018) Somatic SLC35A2 variants in the brain are associated with intractable neocortical epilepsy. Ann Neurol 83:1133-1146

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