Publications by authors named "Lawrence Wrabetz"

93Publications

Brainstem development requires galactosylceramidase and is critical for pathogenesis in a model of Krabbe disease.

Nat Commun 2020 10 23;11(1):5356. Epub 2020 Oct 23.

Hunter James Kelly Research Institute, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo (SUNY), Buffalo, NY, 14214, USA.

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October 2020

Macrophages Expressing GALC Improve Peripheral Krabbe Disease by a Mechanism Independent of Cross-Correction.

Neuron 2020 07 5;107(1):65-81.e9. Epub 2020 May 5.

Hunter James Kelly Research Institute, Departments of Biochemistry and Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY 14203, USA. Electronic address:

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July 2020

Sustained Expression of Negative Regulators of Myelination Protects Schwann Cells from Dysmyelination in a Charcot-Marie-Tooth 1B Mouse Model.

J Neurosci 2018 05 2;38(18):4275-4287. Epub 2018 Apr 2.

Division of Genetics and Cell Biology, San Raffaele Scientific Institute, DIBIT, Milan, Italy 20132,

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May 2018

Consensus guidelines for newborn screening, diagnosis and treatment of infantile Krabbe disease.

Orphanet J Rare Dis 2018 02 1;13(1):30. Epub 2018 Feb 1.

Wadsworth Center, New York State Department of Health, Newborn Screening Program, David Axelrod Institute, 120 New Scotland Ave., Albany, NY, 12201, USA.

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February 2018

Impairment of protein degradation and proteasome function in hereditary neuropathies.

Glia 2018 02 27;66(2):379-395. Epub 2017 Oct 27.

Hunter James Kelly Research Institute and Departments of, Biochemistry and Neurology, Jacobs School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, New York.

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February 2018

Slick (Kcnt2) Sodium-Activated Potassium Channels Limit Peptidergic Nociceptor Excitability and Hyperalgesia.

J Exp Neurosci 2017 14;11:1179069517726996. Epub 2017 Sep 14.

Neuroscience Program, The State University of New York - University at Buffalo, Buffalo, NY, USA.

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September 2017

Acetyl-CoA production from pyruvate is not necessary for preservation of myelin.

Glia 2017 10 28;65(10):1626-1639. Epub 2017 Jun 28.

Hunter James Kelly Research Institute, University at Buffalo, Buffalo, New York, 14203.

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October 2017

Laminin 211 inhibits protein kinase A in Schwann cells to modulate neuregulin 1 type III-driven myelination.

PLoS Biol 2017 Jun 21;15(6):e2001408. Epub 2017 Jun 21.

Hunter James Kelly Research Institute, Department of Biochemistry and Neurology, Jacobs School of Medicine and Biomedical Sciences, The State University of New York at Buffalo, Buffalo, New York, United States of America.

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June 2017

Axon degeneration: Linking axonal bioenergetics to myelin.

J Cell Biol 2016 Nov;215(4):437-440

Hunter James Kelly Research Institute, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY 14203

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November 2016

Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency.

J Neurosci Res 2016 12 18;94(12):1520-1530. Epub 2016 Jul 18.

Department of Anatomy and Cell Biology, College of Medicine, University of Illinois at Chicago, Chicago, Illinois.

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December 2016

Metabolic profiling reveals biochemical pathways and potential biomarkers associated with the pathogenesis of Krabbe disease.

J Neurosci Res 2016 11;94(11):1094-107

Hunter James Kelly Research Institute, Jacobs School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, New York.

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November 2016

Can psychosine and galactocerebrosidase activity predict early-infantile Krabbe's disease presymptomatically?

J Neurosci Res 2016 11;94(11):1084-93

Hunter James Kelly Research Institute, School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York.

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November 2016

YAP and TAZ control peripheral myelination and the expression of laminin receptors in Schwann cells.

Nat Neurosci 2016 07 6;19(7):879-87. Epub 2016 Jun 6.

Department of Biochemistry, Hunter James Kelly Research Institute, Jacobs School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, New York, USA.

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July 2016

Perk Ablation Ameliorates Myelination in S63del-Charcot-Marie-Tooth 1B Neuropathy.

ASN Neuro 2016 Mar-Apr;8(2). Epub 2016 Apr 19.

Hunter James Kelly Research Institute, University at Buffalo, NY, USA Department of Biochemistry, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, NY, USA Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, NY, USA

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December 2016

Altered Trafficking and Processing of GALC Mutants Correlates with Globoid Cell Leukodystrophy Severity.

J Neurosci 2016 Feb;36(6):1858-70

Hunter James Kelly Research Institute and Departments of Biochemistry and Neurology, Jacob School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, New York 14203.

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February 2016

Microprocessor complex subunit DiGeorge syndrome critical region gene 8 (Dgcr8) is required for schwann cell myelination and myelin maintenance.

J Biol Chem 2015 Oct 13;290(40):24294-307. Epub 2015 Aug 13.

From the Department of Neurology and Center for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611 and

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October 2015

Disease specific therapies in leukodystrophies and leukoencephalopathies.

Mol Genet Metab 2015 Apr 7;114(4):527-36. Epub 2015 Feb 7.

Department of Neurology, Children's National Health System, Washington, DC, USA; Center for Genetic Medicine Research, Children's National Health System, Washington, DC USA; Department of Integrated Systems Biology, George Washington University School of Medicine, Washington, DC, USA. Electronic address:

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April 2015

Perlecan is recruited by dystroglycan to nodes of Ranvier and binds the clustering molecule gliomedin.

J Cell Biol 2015 Feb;208(3):313-29

Division of Genetics and Cell Biology, San Raffaele Hospital, 20132 Milan, Italy Department of Biochemistry and Department of Neurology, Hunter James Kelly Research Institute, School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY 14203 Department of Biochemistry and Department of Neurology, Hunter James Kelly Research Institute, School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY 14203

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February 2015

Proteolipid protein cannot replace P0 protein as the major structural protein of peripheral nervous system myelin.

Glia 2015 Jan 28;63(1):66-77. Epub 2014 Jul 28.

Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio.

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January 2015

The Gdap1 knockout mouse mechanistically links redox control to Charcot-Marie-Tooth disease.

Brain 2014 Mar 29;137(Pt 3):668-82. Epub 2014 Jan 29.

1 Institute of Molecular Health Sciences, Cell Biology, Department of Biology, ETH Zurich, Swiss Federal Institute of Technology, Switzerland, ETH-Hönggerberg, 8093 Zürich, Switzerland.

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March 2014

Deletion of GABA-B receptor in Schwann cells regulates remak bundles and small nociceptive C-fibers.

Glia 2014 Apr 29;62(4):548-65. Epub 2014 Jan 29.

Dipartimento di Scienze Farmacologiche e Biomolecolari, Università degli Studi di Milano, Via Balzaretti 9, 20133, Milan, Italy; Blond McIndoe Laboratories, The University of Manchester, Institute of Inflammation and Repair, M13 9PT, Manchester, United Kingdom.

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April 2014

Jab1 regulates Schwann cell proliferation and axonal sorting through p27.

J Exp Med 2014 Jan 16;211(1):29-43. Epub 2013 Dec 16.

Institute of Experimental Neurology (INSPE), Division of Neuroscience; 2 Department of Neurology; and 3 Division of Immunology, Transplantation, and Infectious Disease; San Raffaele Scientific Institute, 20132 Milan, Italy.

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January 2014

α6β1 and α7β1 integrins are required in Schwann cells to sort axons.

J Neurosci 2013 Nov;33(46):17995-8007

San Raffaele Scientific Institute, Milano 20132, Italy, Hunter James Kelly Research Institute, University at Buffalo, State University of New York, New York 14203, Development and Stem Cells Program, Institut de Génétique et de Biologie Moléculaire et Cellulaire, Centre National de la Recherche Scientifique, Unité Mixte de Recherche 7104, Institut National de la Santé et de la Recherche Médicale U964, Université de Strasbourg, Illkirch 67404, France, Department of Medicine, Children's Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, Biomedical Research Centre, School of Biological Sciences, University of East Anglia, Norwich NR4 7TJ, United Kingdom, and Robert Wood Johnson Medical School, Piscataway, New Jersey, New Jersey 08854.

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November 2013

DDIT4/REDD1/RTP801 is a novel negative regulator of Schwann cell myelination.

J Neurosci 2013 Sep;33(38):15295-305

Dulbecco Telethon Institute at Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, 20132 Milan, Italy, UniSR, Vita Salute San Raffaele University, 20132 Milan, Italy, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, 20132 Milan, Italy, Hunter James Kelly Research Institute, University at Buffalo, State University of New York, Buffalo, New York 14203, Quark Pharmaceuticals, 70400 Ness Ziona, Israel, and The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205.

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September 2013

Resetting translational homeostasis restores myelination in Charcot-Marie-Tooth disease type 1B mice.

J Exp Med 2013 Apr 1;210(4):821-38. Epub 2013 Apr 1.

Division of Genetics and Cell Biology and 2 Division of Neuroscience, San Raffaele Scientific Institute, DIBIT, 20132 Milan, Italy.

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April 2013

Endothelial β4 integrin is predominantly expressed in arterioles, where it promotes vascular remodeling in the hypoxic brain.

Arterioscler Thromb Vasc Biol 2013 May 7;33(5):943-53. Epub 2013 Mar 7.

Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 N Torrey Pines Rd, La Jolla, CA 92037, USA.

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May 2013

Selective knockdown of mutant SOD1 in Schwann cells ameliorates disease in G85R mutant SOD1 transgenic mice.

Neurobiol Dis 2012 Oct 2;48(1):52-7. Epub 2012 Jun 2.

Department of Neurology, The University of Chicago Medical Center, 5841 S. Maryland Avenue, Chicago, IL 60637, USA.

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October 2012

Genetic interaction between MTMR2 and FIG4 phospholipid phosphatases involved in Charcot-Marie-Tooth neuropathies.

PLoS Genet 2011 Oct 20;7(10):e1002319. Epub 2011 Oct 20.

Human Inherited Neuropathies Unit, INSPE-Institute for Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy.

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October 2011

TACE (ADAM17) inhibits Schwann cell myelination.

Nat Neurosci 2011 Jun 12;14(7):857-65. Epub 2011 Jun 12.

Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy.

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June 2011

P0S63del impedes the arrival of wild-type P0 glycoprotein to myelin in CMT1B mice.

Hum Mol Genet 2011 Jun 1;20(11):2081-90. Epub 2011 Mar 1.

Present address: Department of Neurodegenerative Disease, Institute of Neurology, UCL, Queen Square, London WC1N 3BG, UK.

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June 2011

Actin polymerization is essential for myelin sheath fragmentation during Wallerian degeneration.

J Neurosci 2011 Feb;31(6):2009-15

Department of Physiology, Mitochondria Hub Regulation Center, College of Medicine, Dong-A University, Busan 602-714, South Korea.

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February 2011

P0 (protein zero) mutation S34C underlies instability of internodal myelin in S63C mice.

J Biol Chem 2010 Dec 11;285(53):42001-12. Epub 2010 Oct 11.

Biology Department, Boston College, Chestnut Hill, Massachusetts 02467-3811, USA.

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December 2010

MicroRNA-deficient Schwann cells display congenital hypomyelination.

J Neurosci 2010 Jun;30(22):7722-8

Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA.

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June 2010

Signals to promote myelin formation and repair.

Nat Rev Neurol 2010 May 20;6(5):276-87. Epub 2010 Apr 20.

Division of Neuroscience and INSPE, San Raffaele Scientific Institute, DIBIT, Milan, Italy.

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May 2010

SCAP is required for timely and proper myelin membrane synthesis.

Proc Natl Acad Sci U S A 2009 Dec 30;106(50):21383-8. Epub 2009 Nov 30.

Department of Molecular and Cellular Neurobiology, Center for Neurogenomics and Cognitive Research, Neuroscience Campus Amsterdam, VU University, 1081 HV Amsterdam, The Netherlands.

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December 2009

CHOP and the endoplasmic reticulum stress response in myelinating glia.

Curr Opin Neurobiol 2009 Oct 8;19(5):505-10. Epub 2009 Sep 8.

Wayne State University, 3217 Scott Hall, 540 E Canfield, Detroit, MI, USA.

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October 2009

Myelin under stress.

J Neurosci Res 2009 Nov;87(15):3241-9

San Raffaele Scientific Institute, DIBIT, Milan, Italy.

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November 2009

Molecular mechanisms of inherited demyelinating neuropathies.

Glia 2008 Nov;56(14):1578-89

The University of Pennsylvania Medical School, Philadelphia, Pennsylvania.

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November 2008

c-Jun is a negative regulator of myelination.

J Cell Biol 2008 May;181(4):625-37

Department of Anatomy and Developmental Biology, University College London, London WC1E 6BT, England, UK.

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May 2008

Active gene repression by the Egr2.NAB complex during peripheral nerve myelination.

J Biol Chem 2008 Jun 2;283(26):18187-97. Epub 2008 May 2.

Molecular and Cellular Pharmacology Training Program, Department of Comparative Biosciences, Graduate Program in Cellular and Molecular Biology, University of Wisconsin, Madison, WI 53706, USA.

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June 2008

Basal lamina: Schwann cells wrap to the rhythm of space-time.

Curr Opin Neurobiol 2006 Oct 7;16(5):501-7. Epub 2006 Sep 7.

San Raffaele Scientific Institute, Dipartimento di Ricerca Biologica e Tecnologica, Via Olgettina 58, Milano, Italy.

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October 2006

In vivo detection of Egr2 binding to target genes during peripheral nerve myelination.

J Neurochem 2006 Sep;98(5):1678-87

Graduate Program in Cellular and Molecular Biology, University of Wisconsin, Madison, Wisconsin, USA.

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September 2006

TGFbeta type II receptor signaling controls Schwann cell death and proliferation in developing nerves.

J Neurosci 2006 Aug;26(33):8417-27

Department of Anatomy and Developmental Biology, University College London, London WC1E 6BT, United Kingdom.

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August 2006

Role of integrins in peripheral nerves and hereditary neuropathies.

Neuromolecular Med 2006 ;8(1-2):191-204

San Raffaele Scientific Institute, DIBIT, Via Olgettina 58, 20132 Milano, Italy.

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March 2007

Direct regulation of myelin protein zero expression by the Egr2 transactivator.

J Biol Chem 2006 Mar 22;281(9):5453-60. Epub 2005 Dec 22.

Molecular and Cellular Pharmacology Program, School of Veterinary Medicine, University of Wisconsin, 2015 Linden Drive, Madison, WI 53705, USA.

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March 2006

Laminins and their receptors in Schwann cells and hereditary neuropathies.

J Peripher Nerv Syst 2005 Jun;10(2):128-43

San Raffaele Scientific Institute, DIBIT 4A2, Via Olgettina 58, 20132 Milan, Italy.

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June 2005

Schwann cell-specific ablation of laminin gamma1 causes apoptosis and prevents proliferation.

J Neurosci 2005 May;25(18):4463-72

Laboratory of Neurobiology and Genetics, The Rockefeller University, New York, New York 10021, USA.

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May 2005

Regulation of cholesterol/lipid biosynthetic genes by Egr2/Krox20 during peripheral nerve myelination.

J Neurochem 2005 May;93(3):737-48

Molecular and Cellular Pharmacology Training Program, University of Wisconsin-Madison, Madison, Wisconsin 53706, USA.

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May 2005

Axonal signals and oligodendrocyte differentiation.

Neurochem Res 2004 May;29(5):979-88

San Raffaele Scientific Institute, DIBIT, Via Olgettina 58, 20132 Milan, Italy.

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May 2004

Expression of laminin receptors in schwann cell differentiation: evidence for distinct roles.

J Neurosci 2003 Jul;23(13):5520-30

Neuropathology Unit, Department of Neuroscience and DIBIT, San Raffaele Scientific Institute, 20132 Milan, Italy.

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July 2003

Peripheral Nerve Dysmyelination Due to P Glycoprotein Overexpression Is Dose-Dependent.

Ann N Y Acad Sci 1999 Oct;883(1):294-301

Department of Neurology and DIBIT, San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milano, ItalyDepartment of Pathobiological Sciences, Waisman Center, University of Wisconsin-Madison, Madison, Wisconsin 53705, USA.

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October 1999

P-Cre Transgenic Mice for Inactivation of Adhesion Molecules in Schwann Cells.

Ann N Y Acad Sci 1999 Oct;883(1):116-123

Department of Neurology and DIBIT, San Raffaele Scientific Institute, via Olgettina 58, 20132 Milano, ItalyDepartment of Pathobiological Sciences, Waisman Center, University of Wisconsin-Madison, Madison, Wisconsin 53705, USA.

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October 1999