Grants Funded by the A-T Children's Project
2011 - 2012
A-T Clinical Center
Johns Hopkins University
Linking Neurodegeneration to Protein Turnover in Ataxia Telangiectasia
Shyamal D. Desai, PhD - LSU-Health Sciences Center-School of Medicine
PCD Mice as an Animal Model of Ataxia in A-T
Kamran Khodakhah, PhD - Albert Einstein College of Medicine
Investigating Connectivity and Neuroinflammation within Corticomotor Networks in Ataxia-Telangiectasia: Improving our Understanding of the Clinical Phenotype
Associate Professor Stephen Rose, University of Queensland, Dr. Kate Sinclair, Royal Children's Hospital, Professor Martin Lavin, University of Queensland
Inspiratory Muscle Training in Patients with Ataxia-Telangiectasia
Emma Ross, PhD - University of Brighton
Addressing Neurological Dysfunction in Ataxia Telangiectasia: Investigation into Novel, Physiologic ATM Activation in Neurons and ATM-PP1 Signaling in Reorganization and Refinement of Post Synaptic Architecture
Benjamin Siddoway PhD - Health Science Center
Developing ATM Conditional Knockout Human Embryonic Stem Cells Into a Disease Model for A-T Neurodegeneration
Yang Xu, PhD - University of California, San Diego
2009 - 2010
A-T Clinical Center
Johns Hopkins University
Non-Traditional Roles of ATM in Neurons
Karl Herrup, Ph.D. - Case Western Reserve University School of Medicine
Maintenance of Congenic Mice
Michael M. Weil, Ph.D. – Colorado State University
Systemic Inflammation in Patients with Ataxia Telangiectasia
Sharon McGrath, MD - Johns Hopkins School of Medicine
2008
A-T Clinical Center
Johns Hopkins University
Impact of Growth Factor (GF) Supplementation on Growth Retardation and Neurodegeneration in Ataxia telangiectasia
Ralf Schubert, PhD - Klinikum der Johann Wolfgang Goethe Universität
Increasing A-T Cell Viability by Pharmacologic Modulation of Intracellular Nicotinamide Adenine Dinucleotide Levels
Rodney Shackelford, DO, PhD - Johns Hopkins University and Howard Lederman, MD, PhD - Johns Hopkins University
Generation of a Panel of Monoclonal Antibodies Against the Human and Mouse ATM Proteins
Yossi Shiloh, PhD - Tel Aviv University
Signaling Pathways Involved in Oxidative Stress-mediated Neurodegeneration in ATM Gene Deficiency
Paul Wong, PhD - MD Anderson Cancer Center
ATM, a Novel Activator of Akt that Regulates Neuronal Survival in Response to Insulin and IGF-1
Da-Qing Yang, Ph.D. - The University of South Dakota
2007
A-T Clinical Center
Johns Hopkins University
Non-Traditional Roles of ATM in Neurons
Karl Herrup, Ph.D. - Case Western Reserve University School of Medicine
Functional Dissection of an ATM-CREB Signaling Pathway in the Nervous System
Randal Tibbetts - U. of Wisconsin, Madison
Quantitative Proteomic Analysis of Cerebrospinal Fluid (CSF) from Ataxia-telangiectasia Patients Using LC/MS-based Label-free Protein Quantification Method
Mu Wang, Ph.D. - Indiana University School of Medicine
Gait Analysis in A-T Mice
Michael M. Weil, Ph.D. - Colorado State University and Mouse Specifics, Inc.
Generation of Disease-Specific Human Embryonic Stem Cells to Study the Mechanism of Pathogeneses in Ataxia-telangiectasia
Yang Xu, Ph.D. - University of California San Diego
A-T Clinical Center
Johns Hopkins University
ATM in Immune Responses
Jessamyn Bagley, PhD - Brigham and Women's Hospital
The Role of the DNA Damage Response in Cerebellar Degeneration of A-T
Ari Barzilai, PhD - Tel Aviv University
Identification and Characterization of Chemicals that Readthrough PTC Mutations in the ATM Gene
Richard A. Gatti, M.D. - UCLA School of Medicine
The role of pro-apoptotic BID as an ATM effector in the DNA-damage response
Atan Gross, Ph.D. - Weizmann Institute of Science
Genome (Chromosome) Instability in the Brain and Neuronal Death in Ataxia Telangiectasia
Prof. Yuri B. Yurov - Russian Academy of Medical Sciences
2005
A-T Clinical Center
Johns Hopkins University
Role of ATM in the Control and Execution of Apoptosis: Crosstalk between ATM and Abl Kinases and Caspases
Daniela Barilá, PhD - Dulbecco Telethon Institute at University of Tor Vergata
Down Regulation of Nucleophosmin (NPM): A New Mechanism to Activate p53 in AT Cells
France Carrier, PhD - University of Maryland, Baltimore
Perinatal Implantation of Human Glial Progenitor Cells as a Treatment Strategy for the Childhood Myelin Disorders
Steven A. Goldman, PhD - Cornell University
The Zebrafish as a Novel Model System of Ataxia-Telangiectasia and Other Related Diseases
Shuji Kishi, M.D., PhD - Instructor in Pathology, Harvard Medical School
Correction of the Neurological Defect in Atm gene-disrupted Mice by the Insoindolin Nitroxide, 5 carbocy-1,1,3,3-tetramethylisoindoline-2-yloxyl (CTMIO)
Martin F. Lavin, PhD - Queensland Institute of Medical Research
Regulation of ATM Pathways by Oncogenic Phosphatase PPM1D
Xiongbin Lu, PhD - Baylor College of Medicine
Lung Function in Ataxia-Telangiectasia
Sharon McGrath, MD - Johns Hopkins School of Medicine
Relationship Between DNA Damage Detection and Signaling Revealed in Humanized Mouse Models of AT and NBS
Andre Nussenzweig, PhD - NIH, NCI
Functional Interactions between ATM,E2F1 and the M/R/N DNA repair complex.
Tanya T. Paull, PhD - The University of Texas at Austin
Exploration of the Function of ATM in Glial Biology
Prithi Rajan, PhD - The Burnham Institute
Iron Chelators as a Pharmacological Treatment to Reduce Spontaneous dsDNA Breaks in Ataxia-Telangiectasia Cells
Rodney Shackelford - Louisiana State University at Shreveport
Aberrant Regulation of Mitochondrial DNA in Ataxia-telangiectasia
Gerald S. Shadel, PhD - Yale University School of Medicine
Understanding ATM
Yossi Shiloh, PhD - Tel Aviv University
Multimodal Stem Cell Action in Inherited CNS Disease
Evan Snyder, MD, PhD - The Burnham Institute
Cell Cycle and Cell Death in atm-Deficient Neuron
Yan Yang, MD, PhD - Case Western Reserve University School of Medicine
2004
A-T Clinical Center
Johns Hopkins University
Gene Therapy for Ataxia-Telangiectasia
Maria Luisa Cortes, PhD - Massachusetts General Hospital
Generation of a Rat Model for Ataxia-Telangiectasia
Martin F. Lavin, PhD - Queenslands Institute of Medical Research
and Michael M. Weil, PhD - Colorado State University
ATM Activates the Myocyte Enhancer Factor-2 (MEF2) Family of Transcription Factors Implicated in Regulation of Neuronal Differentiation and Survival
Stuart Lipton, MD, PhD - The Burnham Institute
The Function of ATM in Neuronal Differentiation: Identification of Targets for High Throughput Screening
Brendan Price, PhD - Dana-Farber Cancer Institute
ATM Gene Transfer for Ataxia-Telangiectasia by a Novel Herpes Amplicon Vector
Suming Wang, MD, PhD
Understanding ATM
Yossi Shiloh, PhD - Tel Aviv University
2003
A-T Clinical Center
Johns Hopkins University
The Role of Atm Protein in the DNA Damage Response in Cerebellar Neuronal Cells
Ari Barzilai, PhD - Tel Aviv University
New Mechanisms to Activate p53 Function in A-T Cells
France Carrier, PhD - University of Maryland
Induction for Hematopoietic Chimerism for Treatment of Immune System Defects in Ataxia-Telangiectasia
John Iacomini, PhD - Massachusetts General Hospital
ATM Modulation of CREB Phosphorylation in the Developing Thymus, Testes and Cerebellum
Wenan Qiang, PhD - Northwestern University Feinberg School of Medicine
Understanding ATM
Yossi Shiloh, PhD - Tel Aviv University
Developing PARP Inhibitors that can Penetrate the Blood Brain Barrier by Oral Dosing
Jir Zhang, PhD - Guilford Pharmaceuticals, Inc.
Desferrioxamine as a Pharmacological Treatment to Increase the Life-Span of Ataxia-Telangiectasia Mice
Suming Wang, MD, PhD
Cell Cycle Events in Ataxia-Telangiectasia: Human and Mouse
Yan Yang, MD, PhD
The Role of ATM in the Mitochondrial Pathway of Apoptosis
Leman Yel, MD - University of California, Irvine
2002
A-T Clinical Center
Johns Hopkins University
Pilot Study: Evaluating the Relative Radiation Sensitivity of ATM Functional & ATM Inactive Human Cell Lines After Treatment With Small-Molecule Modulators
SRI International
Glucocorticoid Mimics Functional ATM Kinase to Prevent Thymic Lymphoma Development in Atm -/- Mice
Mingshan Yan, MD - University of Texas MD Anderson Cancer Center
A Drosophila Model for Ataxia-Telangiectasia
Shelagh Diane Campbell, PhD - University of Alberta
Understanding ATM
Yossi Shiloh, PhD - Tel Aviv University
Induction for Hematopoietic Chimerism for Treatment of Immune System Defects in Ataxia-Telangiectasia
John Iacomini, PhD - Massachusetts General Hospital
Creation of a Transgenic Porcine Model of A-T
Christopher Counter, PhD - Duke University
A Novel Alternative Model System for Identifying Candidate Therapeutics for Ataxia-Telangiectasia
Shuji Kishi, MD, PhD - Dana-Farber Cancer Institute
Experimental Gene Therapy for Ataxia-Telangiectasia
Xandra O. Breakefield, PhD - Massachusetts General Hospital
Effect of EUK - 189 on Motor Performance (Rotarod), Oxidative Damage Markers, and Survival in ATM Knockout Mice
M. Flint Beal, MD - Cornell University Medical College
Linking ATM and Breast Cancer
Kevin D. Brown, PhD - Louisiana State University Health Sciences Center
Telomeres, Telomerase and Lifespan of Brain Cells of ATM Null Mice
Tej K. Pandita, PhD - Washington School of Medicine
2001
A-T Clinical Center
Johns Hopkins University
Overlapping Functions of ATM and ATX in Genome and RNA Surveillance
Robert T. Abraham, PhD - Burnham Institute
Molecular Basis of Pleiotropic Phenotypes of Ataxia-Telangiectasia
Jun Qin, PhD - Baylor College of Medicine
Understanding ATM - Multi-year Grant
Yossi Shiloh, PhD - Tel Aviv University
Strain Background Effects on Atm Nullizygosity
Michael M. Weil, PhD - University of Texas MD Anderson Cancer Center
Identification of Novel ATM-RAD 17 Associated Proteins that Function as Regulators or Downstream Targets
Xiao-Fan Wang, PhD - Duke University Medical Center
Telomeres, Telomerase and Lifespan of Brain Cells of ATM Null Mice
Tej K. Pandita, PhD - Columbia University
Effect of loss of ATM on Glial Cell Function
Martin F. Lavin, PhD - Queenslands Institute of Medical Research
2000
A-T Clinical Center
Johns Hopkins University
Experimental Gene Therapy for A-T Using HSV Amplicon Vectors
Xandra Breakefield, PhD - Massachusetts General Hospital
ATM Gene Transfer for Ataxia-Telangiectasia by a Novel Herpes Amplicon Vector
Suming Wang, MD, PhD - Human Gene Therapy Research Institute
Development of DNA Diagnostic Test for the Ataxia-Telangiectasia (ATM) Gene
A-T Clinical Center at Johns Hopkins University
Neurologic Pathophysiology of Ataxia-Telangiectasia
A-T Clinical Center at Johns Hopkins University
Understanding ATM - Multi-year Grant
Yossi Shiloh, PhD - Tel Aviv University
Molecular Role of Oxidative Stress in Cerebellar Degeneration in A-T
Ari Barzilai, PhD - Tel Aviv University
Mouse Ataxia-Telangiectasia Intervention Study
M. Flint Beal, MD - Cornell University Medical College
Ataxia-Telangiectasia: An Assessment of Global Biochemical Abnormalities
David S. Lawrence, PhD - Albert Einstein College of Medicine
Production of ATM Gene-Targeted Pigs and Cattle by Nuclear Transfer from Cultured Fibroblast Cells
Steven Stice, PhD - University of Georgia
1999
A-T Clinical Center
Johns Hopkins University
Neural Autoantibodies in the Sera of A-T Patients
Robert B. Darnell, MD, PhD - Rockefeller University
Mechanisms of Cytokine-Mediated Neuroprotection in ATM Mutant Mice
Lorise C. Gahring, PhD - University of Utah School of Medicine
Regulation of the Aspergillus DNA Damage Response by Suppressors of ATM Kinase Mutations
Steven D. Harris, PhD - University of Connecticut Health Center
ATR-Activating Therapy for Ataxia-Telangiectasia
Robert T. Abraham, PhD - Duke University Medical Center
Ataxia-Telangiectasia: Activation of Cytoprotective Signaling Pathways
David S. Lawrence, PhD - The Albert Einstein College of Medicine
Neural Stem Cell Transplantation in Animal Models of A-T
Evan Y. Snyder, MD, PhD - Children’s Hospital Boston
Generation of Immunological Reagents to Atm, the Mouse Homolog of the ATM Gene Product
Kevin D. Brown, PhD - Louisiana State University Medical Center
Molecular Basis of Pleiotropic Phenotypes of Ataxia-Telangiectasia
Jun Qin, PhD - Baylor College of Medicine
Development of Improved Protective Strategies Against Free Radical Damage in Ataxia-Telangiectasia
Michael H.L. Green, PhD - University of Brighton
Cloning the Murine Atm Gene
Suming Wang, MD, PhD - Human Gene Therapy Research Institute
A Primate Model for Ataxia-Telangiectasia
Don Wolf, PhD - Oregon Health Sciences University
DNA Damage Signaling in ATM-/- Neurons
Herbert M. Geller, PhD - University of Medicine and Dentistry of New Jersey - Robert Wood Johnson Medical School
1998
A-T Clinical Center
Johns Hopkins University
Ocular Manifestations of Ataxia-Telangiectasia - A Prospective Study
Arman K. Farr, MD - The Wilmer Ophthalmological Institute
Characterization of ATM Kinase Activity
Yossi Shiloh, PhD - Tel Aviv University
Use of Atm Deficient and Atm/Rag2 Deficient Mice to Assess Abnormalities in Eye Movements and Associated Brain Pathology
Carrolee Barlow, MD, PhD - The Salk Institute for Biological Studies
Defects in Cerebellar Purkinje Cell Properties May Underlie Ataxias in Ataxia-Telangiectasia
Peter H. Reinhart, PhD - Duke University Medical Center
Brain Pathology in ATM-deficient Mouse: Correlating Structure and Functions
Menahem Segal, PhD - The Weizmann Institute
Gene Therapy for Ataxia-Telangiectasia by a Novel Herpes Amplicon Vector
Suming Wang, MD, PhD - Human Gene Therapy Research Institute
Defining a Minimal Functional Domain Within the ATM Gene Product
Kevin D. Brown, PhD - Louisiana State University Medical Center
Molecular Mechanism of Cerebellar Degeneration in A-T
Ari Barzilai, PhD - Tel Aviv University
Role of the Extranuclear ATM Protein in Neuronal Function
Martin F. Lavin, PhD - Queensland Institute of Medical Research
Biochemical Analysis of the Ataxia-Telangiectasia Gene Product ATM and Its Relative ATR
Stephen P. Jackson, PhD - University of Cambridge
1997
A-T Clinical Center
Johns Hopkins University
Experiments on Ectopic Expression of the ATM Protein and the Production of Polyclonal and Monoclonal Antibodies Against the ATM Protein
Yossi Shiloh, PhD - Tel Aviv University
ATM in Neurodegeneration
Eva Y.-H.P. Lee, MD, PhD - University of Texas Health Science Center
Role of Purkinje Cell Specific Interacting Partners in ATM Function
Ines Ibanez-Tallon, PhD - The Rockefeller University
Nitric Oxide-Synthesizing Neurons in Pathology of A-T
Rodrigo O. Kuljis, MD - University of Miami School of Medicine
Oxidative Stress and Antioxidants in Ataxia-Telangiectasia
Gerald Cohen, PhD - Mount Sinai School of Medicine
ATM-related Checkpoint Pathway in Saccharomyces cerevisiae
David F. Stern, PhD - Yale University School of Medicine
Contribution of Reactive Oxygen Species to Neuronal Vulnerability in Ataxia-Telangiectasia
Laura L. Dugan, MD - Washington University School of Medicine
Functions of ATM Protein in Neurodevelopment
Rosalind A. Segal, MD, PhD - Beth Israel Deaconess Medical Center
Responses to DNA Damage in Cerebellar Neurons of Atm Mice
Piergiorgio Strata, MD, PhD - University di Torino
Poly (ADP-ribose) Polymerase and Oxidative Stress in Ataxia-Telangiectasia
Joe M. McCord, PhD - University of Colorado Health Sciences Center
Mechanisms of Neuronal Pathogenesis in Ataxia-Telangiectasia
Yang Xu, PhD - University of California, San Diego
1996
A-T Clinical Center
Johns Hopkins University
Translational Regulation of ATM Expression
Orna Elroy-Stein, PhD - Tel Aviv University
Purkinje Cell Development in Genetic Murine Model of Ataxia-Telangiectasia: Effects of Atm Gene Knock-out Purkinje Cell Morphogenesis, Survival and Responsiveness to Neurotrophic Factors
Howard T.J. Mount, PhD - Robert Wood Johnson Medical School
The Mechanism of Neurodegeneration in Ataxia-Telangiectasia
Peter J. McKinnon, PhD - St. Jude Children’s Research Hospital
Purification and Biochemical Analysis of the Ataxia-Telangiectasia Gene Product and Identification of Accessory Proteins
Stephen P. Jackson, PhD - University of Cambridge
A Mouse Model for Ataxia-Telangiectasia
David Baltimore, PhD - Massachusetts Institute of Technology
1995
A-T Clinical Center
Johns Hopkins University
Characterization of the Saccharomyces cerevisiae Homologues, HAT1 and MEC1, of the Human Gene ATM Mutated in Ataxia-Telangiectasia
Dwight M. Morrow, PhD - John Hopkins University
Ataxia-Telangiectasia: From Gene Back to Phenotype
Yossi Shiloh, PhD - Tel Aviv University
MEC1: Understanding its Multiple Functions in Yeast and Relevance to ATM
Ted Weinert, PhD - University of Arizona
The Drosophila mei-41 Gene: An Animal Model for Ataxia-Telangiectasia
Scott Hawley, PhD - University of California at Davis
1994
Complementation of A-T Radioresistant DNA Synthesis by Human Gene Transfer
Timothy J. Jorgensen, PhD - Georgetown University Medical Center
Cloning of genes involved in the induction of neuronal death
Santosh D’Mello, PhD - University of Connecticut
Mechanisms of Neuronal PCD
Eugene M. Johnson, PhD - Washington University Medical School
Lymphocyte Biology and Epstein-Barr Virus Activation in Ataxia-Telangiectasia
Donald E. Mosier, MD, PhD - Scripps Research Institute
Protein Kinase C and the A-T Dependent Response to Ionizing Radiation
Michael Christman, PhD - University of California, San Francisco
Complementation of A-T Groups and A-T Gene Function
Michael B. Kastan, MD, PhD - Johns Hopkins Oncology Center
Localization of the A-T Gene
Aslihan Tolun, PhD - Bogazici University (Collaboration with Patrick Concannon)
1993
Ataxia-Telangiectasia, From Phenotype to Genes
Yossi Shiloh, PhD - Tel Aviv University
Anomalies in Cell Cycle Control in Ataxia-Telangiectasia Post-irradiation
Martin F. Lavin, PhD - Queensland Institute of Medical Research
Characterization of a Candidate Gene for Ataxia-Telangiectasia
Leon Kapp, PhD - SRI International
Isolation and Functional Characterization of the Gene for Ataxia-Telangiectasia
Glen A. Evans, MD, PhD - Salk Insitute for Biological Studies
Cloning the Ataxia-Telangiectasia Gene
Michael Swift, MD - New York Medical College
Isolation of a Gene for Ataxia-Telangiectasia
Richard A. Gatti, MD - UCLA School of Medicine
Isolation of Ataxia-Telangiectasia Genes by cDNA Complementation
Stephen Meyn, MD, PhD - Yale University School of Medicine
Molecular Analysis of Cellular Responses to Ionizing Radiation in Ataxia-Telangiectasia
Kathleen Dixon, PhD - University of Cincinnati