NERIC 2017 Speakers

Matthew E. R. Butchbach, PhD

Matthew E. R. Butchbach, PhD

Head, Motor Neuron Diseases Research Laboratory, Nemours Biomedical Research, University of Delaware
Current Projects

Determine the mechanism(s) of the neuroprotective effect of butyrate compounds on motor neuron diseases
Identification of compounds that increase SMN2 promoter activity and inclusion of exon 7
Development of mathematical models for regulation of SMN2 expression
Characterization of SMN-containing macromolecular complexes in motor neuron processes
Characterization of embryonic stem cell models for SMA
Establishment of a pediatric motor neuron diseases cell collection
Identification of neuroprotective agents against motor neuron excitotoxicity

Selected Publications

Mack SG, DJ Cook, P Dhurjati and MER Butchbach (2014) Systems-based study of cAMP modulation to increase SMN levels for the treatment of spinal muscular atrophy. PLoS ONE 9:e115473.

Maeda M, AW Harris, BF Kingham, CJ Lumpkin, LM Opdenaker, SM McCahan, W Wang and MER Butchbach (2014) Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells. 9:e106818.

Butchbach MER, J Singh, ME Gurney and AHM Burghes (2014) The effect of diet on the protective action of D156844 observed in spinal muscular atrophy mice. Exp Neurol 256:1-6.

He WA, E Berardi, VM Cardillo, S Acharyya, P Aulino, J Thomas-Ahner, J Wang, M Bloomston, P Muscarella, P Nau, N Shah, MER Butchbach, K Ladner, S Adamo, MA Rudnicki, C Keller, D Coletti, F Montanaro and DC Guttridge (2013) NF-κB-mediated Pax7 dysregulation in the muscle microenvironment promotes cancer cachexia. J Clin Invest 123:4821-4835.

Butchbach MER (2011) Trans-splicing, more than meets the eye: multi-faceted therapeutics for spinal muscular atrophy. [commentary] Hum Gene Ther 22:121-125.

Butchbach MER, J Singh, M Þorsteinsdóttir, L Saieva, E Slominski, J Thurmond, T Andrésson, J Zhang, JD Edwards, LR Simard, L Pellizzoni, AHM Burghes and ME Gurney (2010) Evaluation of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy. Hum Mol Genet 19:454-467.

Butchbach MER*, FF Rose Jr*, S Rhoades, J Marston, JT McCrone, R Sinnott and CL Lorson (2010) Effect of diet on the survival and phenotype of a mouse model of spinal muscular atrophy. Biochem Biophys Res Commun 391:835-840. (*these authors contributed equally)

VB Mattis*, MER Butchbach* and CL Lorson (2008) Detection of human survival motor neuron (SMN) protein in mice containing the Smn2 transgene: applicability to preclinical therapy development for spinal muscular atrophy. J Neurosci Methods 175:36-43. (*these authors contributed equally)

Thurmond J, MER Butchbach, M Palomo, B Pease, M Rao, L Bedell, M Keyvan, G Pai, R Mishra, M Haraldsson, T Andresson, G Bragason, M Thosteinsdottir, JM Bjornsson, DD Coovert, AHM Burghes, ME Gurney and J Singh (2008) Synthesis and biological evaluation of novel 2,4-diaminoquinazoline derivatives as SMN2 promoter activators for the potential treatment of spinal muscular atrophy. J Med Chem 51:449-469.

Novoyatleva T, B Heinrich, Y Tang, N Benderska, MER Butchbach, CL Lorson, MA Lorson, C Ben-Dov, P Fehlbaum, L Bracco, AHM Burghes, M Bollen and S Stamm (2008) Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing. Hum Mol Genet 17:52-70.
Burghes AHM and MER Butchbach (2008) Let all DNA vote: who are the amyotrophic lateral sclerosis candidates? [editorial] Neurology 70:662-663.

Gabanella F, MER Butchbach, L Saieva, C Carrissimi, AHM Burghes and L Pellizzoni (2007) Ribonucleoprotein assembly defects correlate with SMA severity and preferentially affect a subset of spliceosomal snRNPs. PLoS ONE 2:e921.

Butchbach MER, JD Edwards and AHM Burghes (2007) Abnormal motor phenotype in the SMND7 mouse model of spinal muscular atrophy. Neurobiol Dis 27:207-219.

Butchbach MER, JD Edwards, KR Schussler and AHM Burghes (2007) A novel method for oral delivery of drug compounds to the neonatal SMND7 mouse model of spinal muscular atrophy. J Neurosci Methods 161:285-290.

Tian G, L Lai, H Guo, Y Lin, MER Butchbach, Y Chang and CG Lin (2007) Translational control of glial glutamate transporter EAAT2 expression. J Biol Chem 282:1727-1737.

Acharyya S, MER Butchbach, Z Sahenk, H Wang, M Saji, M Carathers, MD Ringel, RJE Skipsworth, KCH Fearon, MA Hollingsworth, P Muscarella, AHM Burghes, JA Rafael-Fortney and DC Guttridge (2005) Dystrophin glycoprotein complex dysfunction: a regulatory link between muscular dystrophy and cancer cachexia. Cancer Cell 8:421-432.

Le TT, LT Pham, MER Butchbach*, HL Zhang*, UR Monani, DD Coovert, TO Gavrilina, L Xing, GJ Bassell and AHM Burghes (2005) SMND7, the major product of the centromeric survival motor neuron gene (SMN2), extends survival in mice with spinal muscular atrophy and associates with full-length SMN. Hum Mol Genet 14:845-857. (*these authors contributed equally)

Butchbach MER and AHM Burghes (2004) Perspectives on models of spinal muscular atrophy for drug discovery. Drug Discov Today Dis Models 1:151-156.

Butchbach MER, G Tian, H Guo and CG Lin (2004) Association of excitatory amino acid transporters, especially EAAT2, with cholesterol-rich lipid raft microdomains: importance for EAAT localization and function. J Biol Chem 279:34388-34396.

Guo H*, L Lai*, MER Butchbach*, MP Stockinger*, X Shan, GA Bishop and CG Lin (2003) Increased expression of the glial glutamate transporter EAAT2 modulates excitotoxicity and delays the onset but not the outcome of ALS in mice. Hum Mol Genet 12:2519-2532. (*these authors contributed equally)

Butchbach MER, H Guo and CG Lin (2003) Methyl-b-cyclodextrin but not retinoic acid reduces EAAT3-mediated glutamate uptake and increases GTRAP3-18 expression. J Neurochem 84:891-894.

Guo H, L Lai, MER Butchbach and CG Lin (2002) Human glioma cells and undifferentiated primary astrocytes which express aberrant EAAT2 mRNA inhibit normal EAAT2 protein expression and prevent cell death. Mol Cell Neurosci 21:546-560.

Butchbach MER, L Lai and CG Lin (2002) Molecular cloning, gene structure, expression profile and functional characterization of the mouse glutamate transporter (EAAT3) interacting protein GTRAP3-18. Gene 292:81-90.

Agenda

Wednesday, August 16th

2:00 - 9:00 PM Registration Desk Hours of Operation
(Diamond Foyer)
5:00 - 6:00 PM Reception and Poster Session I Setup
(Promenade)
6:00 - 6:15 PM Welcoming Remarks
(Emerald I-III Ballrooms)

6:15 - 7:30 PM Plated Dinner
(Emerald I-III Ballrooms)
7:30 - 9:00 PM Poster Session I (Undergraduate students and Cores)
(Promenade)

Thursday, August 17th

7:00 AM - 5:30 PM Registration Desk Hours of Operation
(Diamond Foyer)
7:00 - 8:15 AM Breakfast, Career Tables for interested students (Exhibition Hall), Poster Session II Set-up
(Promenade)

  • Tim Hunter, MS, Biotek and Yolanda Nesbeth PhD, Celdara Medical
    "Biotech Career"
  • Corine Farewell, DVM, MBA UVM Innovations
    "Technology Transfer"
  • Charles Irvin, PhD, UVM College of Medicine, Director, Vermont Lung Center, and Ralph Budd, MD, UVM College of Medicine, Director, Vermont Center for Immunology & Infectious Diseases
    "Academic Career"
  • Yanping Liu, MD, PhD, NIH, NIGMS
    "Scientific Careers in Government"
  • Daniel Kolker, PhD, United States Patent and Trademark Office
    “Patent Assessment”
  • Karen Spach, PhD, Senior Medical Writer, Envision Pharma Group
    "Scientific Careers in Government"
8:30 - 9:45 AM Opening Remarks
(Emerald I-III Ballrooms)

NIGMS staff:

  • Yanping Liu, MD, PhD Program Director, NIGMS
    “Overview of Programs in the Center for Research Capacity Building”
  • Arina Kramer, Grants Specialist at the Center for Research Capacity Building, NIGMS
    “IDeA Post Award Management”
9:45 - 10:00 AM Break and Refreshments
(Promenade)
10:00 - 12:00 PM

Concurrent Sessions

  1. Core Presentations of Resources Available to NERIC Investigators
    (Emerald III Ballroom) Chair, Ralph Budd, MD, University Distinguished Professor of Medicine, The University of Vermont Larner College of Medicine

    • Eric Kmiec, PhD, Helen F. Graham Cancer Center & Research Institute, Newark, DE
      "CRISPR-based Gene Editing in Biomedical Research"
    • Youngwook Ahn, PhD, Brown University Transgenics and Gene-Targeting Facility
      “CRISPR/Cas9 Mouse Preparation”
    • Bongsup Cho, PhD, Coordinator INBRE and Professor, The University of Rhode Island
      "Expanding Bioinformatic Core: 3D Printing of Molecules and Viruses!”
    • Nagib Ahsan, PhD, Brown University
      "Proteomics Core Facility"
    • Calvin Vary, PhD, Maine Medical Research Institute
      "Lipidomics"
    • Dwight Matthews, PhD, University of Vermont
      "Metabolomics and 13C-labeling"
  2. Entrepreneurship Workshop: "How to Turn Your Innovative Ideas Into Reality"
    (Emerald II Ballroom)

    • Mercedes Rincón, PhD,   University of Vermont
      "Welcome to the I-Trep program for IDeA states"
    • Keynote speaker, Mathew Portnoy, PhD, Overall HHS SBIR/STTR Program Manager, NIH Program Manager, NIH Office of Extramural Programs
    • Pitch presentations
      • Voot Yin, PhD, MDI Biological Laboratory
      • Qian Chen, PhD, The Warren Alpert Medical School of Brown University/Rhode Island Hospital
    • Round tables (20 minutes each, choose three)
      • Daniel Kolker, PhD, Supervisory Patent Officer, USPTO, and MaryDilys Anderson, PhD, Esq, Finch & Maloney PLLC)
        “Assessing your Invention and Intellectual Property”
      • Samantha Roach-Gerber, VCET, and Erik Monsen, PhD, University of Vermont
        “How To Pitch-How to Sell Your Idea”
      • Cairn Cross, Fresh Tracks Venture
        “Funding Through VC, Angels”
      • Mathew Portnoy, PhD, NIH SBIR Program, and Yolanda Nesbeth, PhD, Celdara Medical
        “Funding Through SBIR/STTR”
      • Sarah O'Connell Kalil, COO, Boston Microfluidics, and Tanya Kanigan, PhD, COO, Genomic Expression Inc.
        “Setting Up Your Startup”
      • Tina Thornton, PhD, University of Vermont
        “I-Trep Program Opportunities”
  3. INBRE Program (Emerald I Ballroom)
    • 10:00 - 11:00 AM - Sharon O'Connor, MS, MBA, Internal Evaluation Manager, Dartmouth College, New Hampshire INBRE
      "INBRE Program Evaluation"
    • 11:00 - 12:00 PM - Elizabeth Ambos, PhD, Executive Officer, Council on Undergraduate Research
      "PM Council on Undergraduate Research Presentation"
10:30 - 11:30 AM COBRE/INBRE Business Grant Managers Meeting with Arina Kramer, Grants Specialist at the Center for Research Capacity Building, NIGMS (Carlton Boardroom)
12:00 - 1:30 PM U.S. Senator Patrick Leahy (Exhibition Hall)

Lunch and Networking
(Exhibition Hall)
VGN Baccalaureate Partner Institution Meeting
(Carlton Boardroom)
1:30 - 3:30 PM

Concurrent Scientific Sessions
Development/Genetics (Emerald I Ballroom) Chair, Bruce Stanton, PhD, Professor of Microbiology and Immunology, Geisel School of Medicine, Dartmouth

  • Featured Speaker: Vicki Losick, PhD, MDI Biological Laboratory
    “Wound Healing the Fruit Fly Way”
  • Featured Speaker: Jodi Camberg, PhD, University of Rhode Island
    "Remodeling protein architectures leads to big changes in cell physiology"
  • Santina Snow, MDI Biological Laboratory
    “The Role of Specific Tissues in Coordinating Changes in Organismal Growth, Longevity and Resilience to Stress in Response to Reduced mRNA Translation in C. Elegans”
  • Aaron Brown, PhD, Maine Medical Center Research Institute
    “A Novel Human Pluripotent Stem Cell-Derived Model of Beige Adipogenesis for Development of Anti-Obesity Therapies”
  • Larissa Williams, PhD, Bates College
    “The Transcription Factor, Nuclear Factor, Erythroid 2 (Nfe2), is a Regulator of the Oxidative Stress Response During Danio Rerio Development”
  • Michaela Reagan, PhD, Tufts University
    “3D Tissue Engineered Design of Bone Marrow Adipose Tissue to Investigate the Influence of Myeloma on Adipocytes and Adipogenesis

Cancer (Emerald III Ballroom) Chair, Mercedes Rincon, PhD, Professor of Medicine, The University of Vermont Larner College of Medicine

  • Featured Speaker: Patrycja Dubielecka, PhD, Brown University
    “Beyond JAK/STAT: Signaling Pathways Contributing to the Pathogenesis of Myeloproliferative Neoplasms”
  • Sonali Barwe, PhD, Alfred I. DuPont Hospital for Children
    “Disseminated Patient-Derived Xenograft Models for Preclinical Testing of New Drugs for Pediatric Leukemia”
  • Mark Perfetto, University of Delaware
    “ADAM9 Cleaves EphrinB1 to Regulate Canonical Wnt Signaling and Colorectal Cancer Cell Migration”
  • Con Sullivan, PhD, University of Maine
    "A Role for the Tumor Necrosis Factor-Alpha-Induced Protein 8-like 1 Gene in Cancer and Inflammation"
  • Sigrid Langhans, PhD, Nemours/Alfred I DuPont Hospital for Children
    “Beta-Hairpin Peptide Hydrogels as Scaffolds for 3D High-Throughput Screening in Drug Discovery”
  • Andrea Villanti, PhD, MPH, Associate Professor, Vermont Center on Behavior and Health, Department of Psychiatry, University of Vermont
    “Characteristics of Young Adult Cigarette Smokers in the U.S.: Findings from the 1st Wave of the PATH Study, 2013-2014”

Infectious Diseases and Immunology (Emerald II Ballroom) Chair, William Green, PhD, Professor of Microbiology and Immunology, Geisel School of Medicine, Dartmouth

  • Featured Speaker: William Landesman, PhD, Green Mountain College
    “Drivers of Variation in theIxodes Scapularis Microbiome”
  • Jason Shapiro, MD, Alpert Medical School, Brown University
    “Immunoglobulin-A Coated Bacteria Represent a Unique Subset of the Microbiome in Patients with Inflammatory Bowel Disease”
  • Md Mahmudul Hasan, University of Vermont
    “Motility of E. Histolytica Through Host Extracellular Matrix: Involvement of Invadosome-like Actin Structures”
  • Rajiv Jumani, University of Vermont
    “Identification and Pharmacokinetic-Pharmacodynamic Modeling of a Piperazine-Based Drug Lead for Cryptosporidiosis”
  • Hacene Boukari, PhD, Delaware State University
    “Tracking HIV Viruses in Human Cervical Mucus”
  • Victoria DeVault, University of Vermont
    “Natural Genetic Variation within a 0.1Mbp Genetic Interval on Chromosome 1 Regulates NKT Cell Development and Function”
3:30 - 3:45 PM Break and Refreshments
(Promenade)
3:45 - 4:45 PM Keynote Lecture I
(Emerald III Ballroom)

  • Jason McLellan, PhD, Geisel School of Medicine at Dartmouth
    “Developing New Therapeutic Interventions to RSV Infection by Inhibiting the Metamorphosis of the RSV Fusion Glycoprotein”
4:45 - 6:00 PM Poster Session II (Graduate students and Postdoctoral Fellows)
(Promenade)
5:30 - 6:30 PM NIH COBRE and INBRE PI Meeting
(Universal Amphitheater)
6:00 PM Dinner on your own in Burlington

Friday, August 18th

8:00 - 9:00 AM Breakfast
(Exhibition Hall)
9:00 - 10:00 AM

Cool ideas from IDeA programs (Emerald III Ballroom) Chair, Charles Irvin, PhD, Professor of Medicine, The University of Vermont Larner College of Medicine

  • Bruce Stanton, PhD, Professor of Microbiology and Immunology, Geisel School of Medicine, Dartmouth
    “Leveraging COBRE Resources with Philanthropy”
  • Marie Stuart, MD, PI of SCD COBRE Program, Director of Hematology Research A.I. DuPont Hospital for Children
    “Single Disease Centered COBREs…A Novel Approach”
  • W. Kelley Thomas, PhD, Professor, Dept. Molecular, Cellular and Biomedical Sciences, University of New Hampshire
    “Early Undergraduate Experiences in Bioinformatics”
10:00 - 12:00 PM

Concurrent Scientific Sessions

Neuroscience (Emerald I Ballroom) Chair, Melissa A. Harrington, PhD, Associate VP for Research, Delaware State University

  • Matthew E. R. Butchbach, PhD, Nemours/Alfred I. duPont Hospital for Children
    "Characterization of Small Molecule Neuroprotectants for Spinal Muscular Atrophy”
  • Featured Speaker: Andrew Kennedy, PhD, Bates College
    "Targeting the Tet Enzymes to Enhance Cognition"
  • Neal Mecum, University of New England
    “Lacrimal Gland Excision in Male and Female C57BL/6 Mice Causes Pain- and Anxiety-like Behavior”
  • Michael Dash, PhD, Middlebury College
    “Synaptic Depotentiation Reduces Sleep Need”
  • Leslie Johnson, PhD, Johnson State College
    “More than Just ‘Baby Weight’: Perceived Postpartum Weight Bias and Well-Being in First-Time Mothers”


Infectious Diseases and Immunology II (Emerald III Ballroom) Chair, Ralph Budd, MD, University Distinguished Professor, Medicine, University of Vermont Larner College of Medicine

  • Michael Secinaro, University of Vermont
    “Glycolysis Primes Effector T Cells for Death Through the Induction of Caspase-3 Activity”
  • Melissa Maginnis, PhD, University of Maine
    “Characterization of Cellular Factors Required for JC Polyomavirus Invasion of Host Cells”
  • Abbas Raza, University of Vermont
    “Sex-Specific Regulation of CNS Autoimmunity by the Signal Lymphocytic Activation Molecule (Slam) Locus”
  • Danielle Dube, PhD, Bowdoin College
    “Discovery of Genes Required for Glycoprotein Biosynthesis in the Gastric Pathogen Helicobacter Pylori”
  • Scott Rusin, Dartmouth College
    “Identification of Candidate CK2 Substrates in Mitosis by Quantitative Phosphoproteomics”

Bioinformatics, Computational Biology, Complex Systems (Emerald II Ballroom) Chair, Cathy Wu, PhD, Edward G. Jefferson Chair of Bioinformatics & Computational Biology, University of Delaware

  • Featured Speaker: Benjamin King, PhD, University of Maine

    "microRNA co-expression network dynamics of Pseudomonas aeruginosa infection in the zebrafish, Danio rerio"
  • Featured Speakers: Dr. Malcolm D’Souza and Dr. Derald Wentzien from Delaware INBRE Wesley College
    “Undergraduate Data Science Research Projects Form an Integral Component of the Wesley College Science and Mathematics Curriculum”
  • Shawn Polson, PhD, University of Delaware
    “Markers to Metagenomes and Back Again: Bioinformatics advances toward understanding microbial viruses”
  • Edward Lyman, PhD, University of Delaware
    “Computational Approaches to GPCR Drug Discovery: The Membrane as a Therapeutic Target”
  • Cathy Wu, PhD, University of Delaware
    “Big Data to Knowledge: Integrative Literature Mining and Panomics Analysis for Kinase Cancer Drug Analytics”
12:00 - 1:00 PM Lunch and Networking
Box lunches
(G's Restaurant)
1:00 PM Conference Concludes