University at Buffalo, The State University of New York

Wrabetz Laboratory

The Wrabetz Laboratory is interested in the molecular genetics of myelination, studied primarily in transgenic mice. They have focused on the pathogenesis of inherited demyelinating neuropathies, and they have developed and characterized multiple mouse models of Charcot-Marie-Tooth (CMT) neuropathies.

Image 02

Wrabetz Research Overview

merge

The Wrabetz Laboratory is interested in the molecular genetics of myelination, studied primarily in transgenic mice. They have focused on the pathogenesis of inherited demyelinating neuropathies, and have developed and characterized multiple mouse models of Charcot-Marie-Tooth (CMT) neuropathies. By introducing authentic CMT mutations from patients into these mice, they are revealing the mechanisms of disease, potential avenues of therapy and the opportunity to test resulting medicines in preclinical trials for CMT. Dr. Wrabetz's laboratory is now translating this experience into the generation of new mouse models of Krabbe disease that carry authentic GalC mutations found in patients with diverse clinical phenotypes. These animal models will provide insight into the effect of different mutations on the function of the galactosylceramidase protein and on the pathogenesis of the disease. Dr. Wrabetz's work has been supported by the National Institutes of Health; Telethon, Italy; the European Community, the Italian and Great Britain Multiple Sclerosis Societies, the Mariani Foundation, the Cariplo Foundation, SUNY Brain Network of Excellence, and the Charcot-Marie-Tooth Association.

HJKRI Lawrence Wrabetz profile picture

Faculty and Staff

Dr. Lawrence Wrabetz
Director, HJKRI
UB Distinguished Professor of Neurology & Biochemistry

Lawrence Wrabetz M.D. is the Director of the Hunter James Kelly Research Institute and Professor in Neurology and Biochemistry in the School of Medicine and Biomedical Sciences, University at Buffalo...

pix

Ghjuvan'Ghjacumu Shackleford

Post Doctoral Research Scientist, HJKRI, Wrabetz Lab

  • 2015 - present: Hunter James Kelly Research Institute, University at Buffalo, NY, USA, Postdoctoral Research scientist
  • 2010 - 2014: PhD in Neuroscience, Paris Descartes University, France. UMR-S 1124 "New Therapeutic for Myelination"
  • 2008 - 2010: Master in cellular and molecular biology and pharmacology, Paris Descartes University, France


  •    

    pix

    Mariapaola Sidoli

    PhD student, HJKRI, Wrabetz Lab

  • 2011 - present: PhD student of University at Buffalo, NY, USA School of medicine and biomedical sciences, Department of Biochemistry, Hunter James Kelly Research Institute
  • 2008 - 2011: Master Degree in Pharmaceutical, veterinary and medical Biotechnology, Faculty of Medicine and Surgery, Universita Vita-Salute San Raffaele, Milan, Italy
  • 2004 - 2008: Bachelor Degree in Biological Science (molecular curriculum), University of Milan-Bicocca, Milan, Italy
  •    

    pix

    Jordan VerPlank

    PhD student, HJKRI, Wrabetz Lab

  • 2007 - 2011: B.S. in Biology, Duquesne University, Pittsburgh, Pennsylvania
  • 2011 - present: Doctoral training, University at Buffalo, Buffalo, New York
  • Students

    pix
    Joseph Abdul-Malak, Undergraduate student, Biomedical Sciences

    Past Members

    • Nicolo' Musner, PhD Student
    • Franscesca Ornaghi, PhD Student
    • Neil Robertson, Undergraduate Student
    • Paola Saveri, Research Technician
    • Domenica Vizzuso, PhD Student
    • Sophie Belin, Post Doctoral Research Scientist

    Selected Publications

    • Das I, Krzyzosiak A, Schneider K, Wrabetz L, D'Antonio M, Barry N, Sigurdardottir A, Bertolotti A.Preventing proteostasis diseases by selective inhibition of a phosphatase regulatory subunit. Science, 348:239-242, 2015.
    • Porrello E, Rivellini C, Dina G, Triolo D, Del Carro U, Ungaro D, Panattoni M, Feltri ML, Wrabetz L, Pardi R, Quattrini A, Previtali SC. Jab1 regulates Schwann cell proliferation and axonal sorting through. J Exp Med, 211:29-43, 2014.
    • D'Antonio M, Musner N, Scapin C, Ungaro D, Del Carro U, Ron D, Feltri ML, Wrabetz L. Resetting translational homeostasis via Inactivation of Gadd34 Restores Myelination in CMT1B Mice. J Exp Med, 210(4), 821-838, 2013.
    • Doddrell RDS, Dun X-P, Shivane A, Feltri ML, Wrabetz L, Wegner M, Sock E, Hanemann CO, Parkinson DB. Loss of Sox10 function contributes to the phenotype of human merlin null Schwannoma cells. Brain, 136:549-63, 2013. PMC3572932.
    • Saporta MAC, Shy BR, Patzko A, Bai Y, Pennuto M, Ferri C, Kirschner D, Avila R, Southwood CM, Gow A, Feltri ML, Wrabetz L*, Shy ME*. MpzR98C arrests Schwann cell development in a mouse model of early-onset Charcot-Marie-Tooth 1B. Brain, 135:2032-47, 2012. (*shared last authorship) PMC3381724
    • Fratta P, Saveri P, Zambroni D, Ferri C, Tinelli E, Messing A, D'Antonio M, Feltri ML, Wrabetz L. P0S63del impedes the arrival of wildtype P0 glycoprotein to myelin in CMT1B mice. Hum Mol Genet, 20: 2081-2090, 2011.
    • La Marca R, Cerri F, Horiuchi K, Bachi A, Feltri ML, Wrabetz L, Blobel CP, Quattrini A, Salzer JL, Taveggia C. ADAM17/TACE inhibits Schwann cell myelination. Nat Neurosci, 14:857-65, 2011.
    • Taveggia C, Feltri ML, Wrabetz L. Signals to promote formation and repair of myelin. Nat Rev Neurol, 6(5):276-287, 2010. PMC3062363
    • Avila RL, D'Antonio M, Bachi A, Inouye H, Feltri ML, Wrabetz L, and Kirschner DA. P0 (Protein Zero) mutation Ser34Cys underlies instability of internodal myelin in transgenic mice. J Biol Chem, 285, 42001-42012, 2010. PMC3009926
    • Woodhoo A, Duran Alonso M, Droggiti A, Turmaine M, D'Antonio M, Parkinson D, Wilton D, Al-Shawi R, Simons P, Shen J, Guillemot F, Radtke F, Meijer D, Feltri ML , Wrabetz L, Mirsky R and Jessen KR. Notch controls embryonic Schwann cell differentiation, postnatal myelination and adult plasticity. Nat Neurosci, 12(7):839-847, 2009.
    • Gow A, and Wrabetz L. CHOP and the endoplasmic reticulum stress response in myelinating glia. Curr Opin Neurobiol, 9(5):505-510, 2009.
    • Pennuto M, Tinelli E, Malaguti MC, Del Carro U, Ron D, D'Antonio M, Quattrini A, Feltri ML, Wrabetz L. Ablation of the UPR-mediator CHOP restores motor function and reduces demyelination in Charcot Marie Tooth 1B mice. Neuron, 57: 393-405, 2008. PMC2267889 (Featured Article Feb 7, 2008 Issue)
    • Scherer SS, Wrabetz L. Molecular Mechanisms of Inherited Demyelinating Neuropathies. Glia, 56:1578-89, 2008. PMC2570024 (Cover Illustration)

    Links