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.


(Information subject to change)

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 (Emerald Promenade)
6:00 - 6:15 PM Welcoming Remarks (Emerald I-III Ballrooms) Ralph Budd, MD, Director, Vermont Center for Immunology and Infectious Diseases, UVM College of Medicine, Judith Van Houten, PhD,  Director, Vermont Genetics Network, UVM President Thomas Sullivan and UVM Provost David Rosowsky, PhD
6:15 - 7:30 PM Plated Dinner (Emerald I-III Ballrooms)
7:30 - 9:00 PM Poster Session I (Undergraduate students and Cores) (Emerald Promenade)

Thursday, August 17th

COBRE/INBRE Business Grant Managers Meeting with Arina Kramer, Grants Specialist at the Center for Research Capacity Building, NIGMS (Carlton Boardroom)
7:00 AM - 5:30 PM Registration Desk Hours of Operation (Diamond Foyer)
7:00 - 8:15 AM Breakfast, Career Tables for interested students, Poster Session II Set-up (Exhibition Hall)
8:30 - 9:15 AM Opening Remarks (Emerald I-III Ballrooms) NIGMS staff:
9:30 - 10:00 AM Break and Refreshments (Emerald Promenade)
10:00 - 12:00 PM Concurrent Sessions
  1. Core Presentations of resources available to NERIC investigators (about 20 min each, followed by table discussions with individual Core leaders) (Emerald III Ballroom)
    • 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
    • Dwight Matthews, PhD, University of Vermont
      "Metabolomics and 13C-labeling"
  2. Entrepreneurship Workshop: how to turn your innovative ideas into reality. (Emerald II Ballroom)
    • Keynote speaker, Mathew Portnoy, PhD, Overall HHS SBIR/STTR Program Manager, NIH Program Manager, NIH Office of Extramural Programs
    • Pitch presentations by IDeA entrepreneurs
    • Mercedes Rincón, PhD,  Associate Professor, Medicine Immunobiology Program, UVM College of Medicine
      "Welcome to the I-Trep program for IDeA states"
    • Three Round tables, topics to choose
  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 Lunch and Networking (Exhibition Hall)

VGN Baccalaureate Partner Institution Meeting (Carlton Boardroom)
1:30 - 3:30 PM Concurrent Scientific Sessions (solicit themes from PIs and from abstracts received). Development/Genetics (Emerald I Ballroom) Chair, Cancer (Emerald III Ballroom) Chair,
  • Featured Speaker: Patrycja Dubielecka, PhD, Brown University
    “Beyond JAK/STAT: Signaling Pathways Contributing to the Pathogenesis of Myeloproliferative Neoplasms”
Infectious Diseases and Immunology (Emerald II Ballroom) Chair, William Green, PhD, Professor of Microbiology and Immunology, Geisel School of Medicine, Dartmouth
3:30 - 3:45 PM Break and Refreshments (Emerald Promenade)
3:45 - 4:45 PM Keynote Lecture I (Emerald III Ballroom)
4:45 - 6:00 PM Poster Session II (Graduate students and Postdoctoral Fellows) (Emerald III Ballroom)
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)
  • 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, Judith Van Houten, PhD
  • Featured Speaker: Matthew E. R. Butchbach, PhD, University of Delaware
    "Characterization of Small Molecule Neuroprotectants for Spinal Muscular Atrophy”

Cardiovascular and Pulmonary Systems (Emerald III Ballroom) Chair, Bruce Stanton, PhD

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
  • 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”
12:00 - 1:00 PM Lunch and Networking Box lunches (G's Restaurant)
1:00 PM Conference Concludes