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  Home > About Lab Medicine and Pathology > Faculty and Staff > Harry T. Orr, Ph.D.

Harry T. Orr, Ph.D.

IHG Orr Harry
Contact Information

Phone: 612-625-3647
Fax: 612-626-7031




2101 6th Street SE
Delivery Code 1241
Wallin Medical Biosciences Building
Minneapolis, MN 55455


Research Interests

Dr. Orr's research program is focused on the molecular genetics of mammalian development and neurodegenerative diseases. He and his colleagues recently cloned the gene for an autosomal dominant form of spinocerebellar ataxia (SCA 1). They showed that the disease is due to expansion of an unstable trinucleotide repeat (CAG) within the SCA 1 gene, which they have mapped to the short arm of human chromosome. They are examining the role of the SCA 1 gene in normal CNS function and how this mutation disrupts this function. The investigators will be working with protein biochemists to characterize the normal and abnormal SCA 1 protein.  In October 2009, Dr. Orr received an NIH Recovery Act grant for his SCA1 work.

Educational Background

  • Oakland University, Rochester, MI (1971), B.A. (Biology)
  • Washington University, St. Louis, MO (1976), Ph.D. (Neurobiology)
  • Harvard University, Cambridge, MA (1980), Research Fellowship (Biochemistry and Molecular Biology)

Professional Background

  • Assistant Professor, University of Minnesota Medical School, Department of Laboratory Medicine and Pathology, 1980 - 1984
  • Associate Professor, University of Minnesota Medical School, Department Laboratory Medicine and Pathology, 1984 - 1989
  • Professor of Pathology, University of Minnesota Medical School, Department of Laboratory Medicine and Pathology, 1989 - present
  • Member, University of Minnesota, Institute of Human Genetics, 1984 - present
  • Professor, University of Minnesota, Biochemistry, 1994 - present
  • Director, University of Minnesota, Institute of Human Genetics, 1998 - 2007
  • Directory, University of Minnesota, Institute for Translational Neuroscience, 2007 - present

Professional Honors

  • Leukemia Society of America Special Fellow, 1979 - 1981
  • Searle Scholar Award, 1981 - 1984
  • Leukemia Society of America Scholar, 1982 - 1987
  • Young Investigator Award, American Soc. of Histocompatibility Immunogenetics, 1985
  • Kilby International Award, 1995
  • MERIT Award, NIH/NIAID, 1995 - 2000
  • Milton Wexler Award for Research in HD, HDSA, 1999
  • Javits Investigator Award, NINDS/NIH, 2004

Professional Memberships

  • NIH, NIAID, Transplantation & Immunogenetics Subcommittee, 1984 - 1988
  • NIH, DRG Member Mammalian Genetics Study Section , 1989 - 1993
  • NIH, NIAID, Ad-hoc Council Member, 1992 - 1992
  • NIH, DRG Member Mammalian Genetics Study Section, 1997 - 2000
  • NIH, DRG, Chair Mammalian Genetics Study Section, 1998 - 2000

Community and University Service

  • Developmental Biology Symposium, 1994
  • Center of Developmental Biology Executive Committee, 1994
  • Organizing Committee Developmental Biology Symposium, 1994
  • Graduate School Health Sciences Research Advisory Committee, 1994
  • University Senate



  • Jorgensen ND, Andresen JM, Lagalwar S, Armstrong B, Stevens S, Byam CE, Duvick LA, Lai S, Jafar-Nejad P, Zoghbi HY, Clark HB, Orr HT. Phosphorylation of ATXN1 at Ser776 in the cerebellum. J Neurochem. 2009 Jul;110(2):675-86.
  • Carlson KM, Melcher L, Lai S, Zoghbi HY, Clark HB, Orr HT. Characterization of the zebrafish atxn1/axh gene family. J Neurogenet. 2009;23(3):313-23.
  • Gao Y, Zu T, Low WC, Orr HT, McIvor RS. Antisense RNA sequences modulating the ataxin-1 message: molecular model of gene therapy for spinocerebellar ataxia type 1, a dominant-acting unstable trinucleotide repeat disease. Cell Transplant. 2008;17(7):723-34.
  • Orr HT. Unstable nucleotide repeat minireview series: a molecular biography of unstable repeat disorders. J Biol Chem. 2009 Mar 20;284(12):7405.
  • Jorgensen ND, Andresen JM, Pitt JE, Swenson MA, Zoghbi HY, Orr HT. Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1. J Neurochem. 2007 Sep;102(6):2040-8.
  • Serra HG, Duvick L, Zu T, Carlson K, Stevens S, Jorgensen N, Lysholm A, Burright E, Zoghbi HY, Clark HB, Andresen JM, Orr HT. RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice. Cell. 2006 Nov 17;127(4):697-708.
  • He Y, Zu T, Benzow KA, Orr HT, Clark HB, Koob MD. Targeted deletion of a single Sca8 ataxia locus allele in mice causes abnormal gait, progressive loss of motor coordination, and Purkinje cell dendritic deficits. J Neurosci. 2006 Sep 27;26(39):9975-82.
  • Vierra-Green CA, Orr HT, Zoghbi HY, Ferrington DA. Identification of a novel phosphorylation site in ataxin-1. Biochim Biophys Acta. 2005 May 15;1744(1):11-8.
  • Riley BE, Zoghbi HY, Orr HT. SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal. J Biol Chem. 2005 Jun 10;280(23):21942-8.
  • Kaytor MD, Byam CE, Tousey SK, Stevens SD, Zoghbi HY, Orr HT. A cell-based screen for modulators of ataxin-1 phosphorylation. Hum Mol Genet. 2005 Apr 15;14(8):1095-105.

For a complete list of Dr. Orr's publications, please see the National Library of Medicine's PubMed Search.

Invited Lectures


  • Research Seminar, Mayo-Jacksonville, FL- March


  • Molecular Pathology Seminar. Johns Hopkins University School of Medicine, Baltimore
  • Neuroscience Institute, University of Tennessee Health Science Center, Memphis
  • Research seminar, Department of Physiology & Biophysics, SUNY at Buffalo
  • Research Seminar, Department of Pathology, Case Western Reserve University, Cleveland, OH
  • Research seminar, Neuroscience Series, NINDS/NIH, Bethesda, MD


  • Program in Neuroscience, University of Massachusetts, Worcester, MA
  • Department of Genetics, Cell Biology Anatomy, University of Nebraska Medical Center, Omaha, NE
  • Department of Neuroscience, University of California – San Diego
  • Biomedical Sciences Program, University of California – San Francisco
  • Research Seminar, Merck Research Labs, Boston, MA


  • Center for Neurogenomics and Cognitive Research (CNCR). VU Medical Center, Amsterdam, Netherlands


  • Department of Pharmacology, University of Washington School of Medicine, Seattle
  • Department of Pathology, Harvard University School of Medicine, Boston
  • Department of Human Genetics, Emory University School of Medicine, Atlanta
  • Children's Memorial Institute for Education & Research, Northwestern University, Chicago
  • University of North Carolina Neuroscience Center, Chapel Hill
  • Neuroscience Research Seminar University of California, Los Angeles
  • Neuroscience Research Seminar University of Illinois, Urbana-Champaign


  • The Jackson Laboratory, Bar Harbor, ME
  • University of Nebraska Health Science Center, Omaha, NE (graduate student invitee-Biochemistry)
  • Research Seminar Arizona State University, Tempe, AZ



  • Invited Speaker, Ataxia Investigators Meeting, Las Vegas, NV – March
  • Plenary Speaker, Academy of Neurology Annual Meeting, Chicago, IL – April
  • Plenary Speaker, European Society of Human Genetics Annual Meeting, Barcelona, Spain – June
  • Invited Speaker, Gordon Research Conference, Neurobiology of Brain Disorders, Oxford, Englan – August
  • Guest Speaker, Ernst Klenk Symposium in Molecular Medicine Protein Aggregation in Health & Disease, Cologne, Germany – September


  • Invited talk – GeNeMove Symposium, Hereditary Movement Disorders, Bonn, Germany
  • Invited talk – Enabling technologies for Alzheimer’s disease, Fidelity Foundation, Bar Harbor, ME
  • Keynote Lecture, Interdisciplinary Meeting: Neurometabolic Diseases and Aging, Ulm, Germany


  • Invited Speaker, Neurodegenerative Diseases - Molecular Mechanisms in a Functional Proteomics Framework. Berlin, Germany
  • Presidential Special Lecture, “Neurodegenerative Disorders: Linking Basic and Clinical Neurosciences”, Society for Neuroscience Annual Meeting, Atlanta
  • Keynote Address, 5th International Conference on Unstable Micorsatellites and Human Disease, Granada Spain


  • Invited Speaker, Ataxia Investigator Meeting, Tampa
  • Invited Speaker, Chicago Chapter, Society for Neuroscience Meeting
  • Plenary Talk, II International Symposium The New Prion Biology: Basic Science, Diagnosis and Therapy. Venice, Italy
  • Invited Speaker, 3rd.Beijing International Symposium on Neurodegenerative Diseases and Stroke, Beijing, China
  • Keynote Keith R. Porter Lecturer, American Society for Cell Biology Summer Meeting on Nuclear Architecture & Disease. Iowa State University
  • Invited Speaker, EMBL Mouse Molecular Genetics Meeting, Heidelberg, Germany
  • Speaker, Symposium: Mechanisms of neuro-degeneration: insights from worms, flies and mice. CHOP/ University of Pennsylvania, Philadelphia
  • Speaker, National Parkinson Foundation 9th International Symposium on Parkinson’s Disease, Washington, DC
  • Co-Chair & Invited Speaker, American Society of Cell Biology 45th Annual Meeting, Minisymposium – Protein misfolding and disease, San Francisco


  • Invited Speaker, 4th International Conference on Unstable Microsatellites & Human Disease”, Banff, Canada
  • Invited Speaker-Gordan Research Conference, “Molecular & Cellular Neurobiology”, Hong Kong, China
  • Keynote Lecture-Hertie-Institute Conference, Building Dedication, Tubingen, Germany
  • Invited Speaker-Sir Edward Youde Memorial Fund Postgraduate Conference, “Model Organism Research & Human Disease”, Hong Kong, China
  • Invited Speaker, Conformational Disorders Symposium, 9th International Conference on Alzheimer’s Disease and Related Disorders, Philadelphia, PA


  • Invited Speaker, Molecular Mechanisms of Neurodegeneration, Milan, Italy
  • Invited participant. NIMH Mouse Models of Fragile X Workshop. Jackson Labs, Bar Harbor, ME
  • Invited Speaker, Symposium in honor of Constantino Sotelo. Catania, Sicily
  • Invited Speaker: Fondation des Treilles Conference, Marseille
  • Platform presentation, Annual meeting-American Society of Human Genetics, Los Angeles, CA

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