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Research Output 1972 2019

Chapter

Spinocerebellar ataxia type 1

Orr, H. T., Dec 1 2007, Neurobiology of Disease. Elsevier Inc., p. 149-155 7 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Spinocerebellar Ataxias
Purkinje Cells
Ataxia
RNA Interference
Glutamine
5 Citations (Scopus)

Spinocerebellar ataxia type 1: Molecular mechanisms of neurodegeneration and preclinical studies

Pérez Ortiz, J. M. & Orr, H. T., Jan 1 2018, Advances in Experimental Medicine and Biology. Springer New York LLC, p. 135-145 11 p. (Advances in Experimental Medicine and Biology; vol. 1049).

Research output: Chapter in Book/Report/Conference proceedingChapter

Spinocerebellar Ataxias
Phosphorylation
Purkinje Cells
Proteins
RNA Splicing
Comment/debate
21 Citations (Scopus)

Dinucleotide repeat polymorphism at the D6S109 locus

Ranum, L. P. W., Chung, M. Y., Duvick, L. A., Zoghbi, H. Y. & Orr, H. T., Mar 11 1991, In : Nucleic acids research. 19, 5, 1 p.

Research output: Contribution to journalComment/debate

Molecular Sequence Data
Dinucleotide Repeats
Chromosomes, Human, Pair 6
Oligodeoxyribonucleotides
Nucleic Acid Repetitive Sequences

Erratum: Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures (Nature (1997) 389 (971-974))

Skinner, P. J., Koshy, B. T., Cummings, C. J., Klement, I. A., Helin, K., Servadio, A., Zoghbi, H. Y. & Orr, H. T., Jan 15 1998, In : Nature. 391, 6664, 1 p.

Research output: Contribution to journalComment/debate

2 Citations (Scopus)

Erratum: The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1 (Nature (1997) 389 (974-978))

Matilla, A., Koshy, B. T., Cummings, C. J., Isobe, T., Orr, H. T. & Zoghbi, H. Y., Feb 19 1998, In : Nature. 391, 6669, 1 p.

Research output: Contribution to journalComment/debate

6 Citations (Scopus)
DNA Repeat Expansion
Frontotemporal Dementia
Poisons
Amyotrophic Lateral Sclerosis
Dementia
Conference contribution

Serial optical coherence scanner for imaging neurodegenerative diseases

Liu, C. J., Rainwater, O., Orr, H. T. & Akkin, T., Jan 1 2018, Clinical and Translational Biophotonics, TRANSLATIONAL 2018. OSA - The Optical Society, Vol. Part F91-TRANSLATIONAL 2018.

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Neurodegenerative diseases
Optical tomography
Brain
Visualization
Tissue
Editorial
29 Citations (Scopus)

Prioritized research recommendations from the National Institute of Neurological Disorders and Stroke Parkinson's Disease 2014 Conference

Sieber, B. A., Landis, S., Koroshetz, W., Bateman, R., Siderowf, A., Galpern, W. R., Dunlop, J., Finkbeiner, S., Sutherland, M., Wang, H., Lee, V. M. Y., Orr, H. T., Gwinn, K., Ludwig, K., Taylor, A., Torborg, C. & Montine, T. J., Oct 1 2014, In : Annals of Neurology. 76, 4, p. 469-472 4 p.

Research output: Contribution to journalEditorial

National Institute of Neurological Disorders and Stroke
Parkinson Disease
Biomedical Research
Research
28 Citations (Scopus)

Structure, Function, and Biosynthesis of the Major Human Histocompatibility Antigens (HLA‐A and HLA‐B)

Krangel, M. S., Orr, H. T. & Strominger, J. L., Jun 1980, In : Scandinavian Journal of Immunology. 11, 6, p. 561-571 11 p.

Research output: Contribution to journalEditorial

Histocompatibility Antigens
Antigens
Human Chromosomes
HLA Antigens
Liposomes
Review article
143 Citations (Scopus)

Beyond the qs in the polyglutamine diseases

Orr, H. T., Apr 15 2001, In : Genes and Development. 15, 8, p. 925-932 8 p.

Research output: Contribution to journalReview article

Cysteine Endopeptidases
Ubiquitins
Multienzyme Complexes
Spinocerebellar Ataxias
Huntington Disease
102 Citations (Scopus)

Cell biology of spinocerebellar ataxia

Orr, H. T., Apr 1 2012, In : Journal of Cell Biology. 197, 2, p. 167-177 11 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Cell Biology
Proteins
Cerebellar Ataxia
Ataxia
54 Citations (Scopus)

Emerging pathogenic pathways in the spinocerebellar ataxias

Carlson, K. M., Andresen, J. M. & Orr, H. T., Jun 1 2009, In : Current Opinion in Genetics and Development. 19, 3, p. 247-253 7 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Genes
Spinocerebellar Degenerations
Cerebellar Diseases
Neurodegenerative Diseases
12 Citations (Scopus)

Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias

Ingram, M. A. C., Orr, H. T. & Clark, H. B., May 1 2012, In : Brain Research Bulletin. 88, 1, p. 33-42 10 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Trinucleotide Repeats
Mutant Proteins
Trinucleotide Repeat Expansion
Ataxia
7 Citations (Scopus)

Genetic approaches to pathogenesis of neurodegenerative diseases

Orr, H. T. & Clark, H. B., Jan 1 1995, In : Laboratory Investigation. 73, 2, p. 161-171 11 p.

Research output: Contribution to journalReview article

Animal Disease Models
Nerve Degeneration
Brain Diseases
Molecular Cloning
Neurodegenerative Diseases
1008 Citations (Scopus)

Glutamine repeats and neurodegeneration

Zoghbi, H. Y. & Orr, H. T., Jun 24 2000, In : Annual Review of Neuroscience. 23, p. 217-247 31 p.

Research output: Contribution to journalReview article

Glutamine
Neurodegenerative Diseases
Trinucleotide Repeat Expansion
Atrophic Muscular Disorders
Spinocerebellar Ataxias
136 Citations (Scopus)

HLA and maternal-fetal recognition

Hunt, J. S. & Orr, H. T., Jan 1 1992, In : FASEB Journal. 6, 6, p. 2344-2348 5 p.

Research output: Contribution to journalReview article

Histocompatibility Antigens
Mothers
Trophoblasts
Embryonic Structures
Genes
12 Citations (Scopus)

Into the depths of ataxia

Orr, H. T., Feb 1 2004, In : Journal of Clinical Investigation. 113, 4, p. 505-507 3 p.

Research output: Contribution to journalReview article

Ataxia
Cerebellar Cortex
Cerebellar Nuclei
Gait
Neurons
5 Citations (Scopus)

Linkage studies in dominantly inherited ataxias.

Zoghbi, H. Y., Frontali, M., Orr, H. T., Sandkuijl, L., Cann, H., Sasaki, H., Chamberlain, S., Terrenato, L. & Rich, S. S., Jan 1 1993, In : Advances in neurology. 61, p. 133-137 5 p.

Research output: Contribution to journalReview article

Spinocerebellar Degenerations
Dominant Genes
Genetic Linkage
Ataxia
8 Citations (Scopus)

Lurcher, nPIST, and autophagy

Orr, H. T., Aug 29 2002, In : Neuron. 35, 5, p. 813-814 2 p.

Research output: Contribution to journalReview article

Autophagy
Neurologic Mutant Mice
Glutamate Receptors
Ion Channels
Glutamic Acid
341 Citations (Scopus)

Major histocompatibility antigens: The human (HLA-A,-B,-C) and murine (H-2K, H-2D) class I molecules

Ploegh, H. L., Orr, H. T. & Strominger, J. L., May 1981, In : Cell. 24, 2, p. 287-299 13 p.

Research output: Contribution to journalReview article

H-2 Antigens
beta 2-Microglobulin
Histocompatibility Antigens
HLA-A Antigens
Histocompatibility Antigens Class II
74 Citations (Scopus)

Maternal/fetal interactions: The role of the MHC class I molecule HLA-G

Schmidt, C. M. & Orr, H. T., Dec 1 1993, In : Critical Reviews in Immunology. 13, 3-4, p. 207-224 18 p.

Research output: Contribution to journalReview article

Molecular Sequence Data
Embryonic and Fetal Development
HLA-G Antigens
Chorion
Histocompatibility Antigens Class I

Molecular genetics of the human leukocyte antigen complex.

Orr, H. T., Jan 1 1989, In : Immunology series. 43, p. 319-338 20 p.

Research output: Contribution to journalReview article

Pseudogenes
Chromosome Mapping
Molecular Cloning
HLA Antigens
Molecular Biology
6 Citations (Scopus)

Nuclear ataxias.

Orr, H. T., May 1 2010, In : Cold Spring Harbor Perspectives in Biology. 2, 5, p. a000786

Research output: Contribution to journalReview article

Ataxia
Cytology
Neurodegenerative diseases
Neurosciences
Cell Nucleus
90 Citations (Scopus)

Polyglutamine diseases: Protein cleavage and aggregation

Zoghbi, H. Y. & Orr, H. T., Oct 1 1999, In : Current opinion in neurobiology. 9, 5, p. 566-570 5 p.

Research output: Contribution to journalReview article

Cell Nucleus
Neurodegenerative Diseases
Proteins
polyglutamine
51 Citations (Scopus)

Polyglutamine neurodegeneration: Expanded glutamines enhance native functions

Orr, H. T., Jun 1 2012, In : Current Opinion in Genetics and Development. 22, 3, p. 251-255 5 p.

Research output: Contribution to journalReview article

Glutamine
Trinucleotide Repeats
Neurodegenerative Diseases
polyglutamine
Proteins
119 Citations (Scopus)

Polyglutamine neurodegenerative diseases and regulation of transcription: Assembling the puzzle

Riley, B. E. & Orr, H. T., Aug 15 2006, In : Genes and Development. 20, 16, p. 2183-2192 10 p.

Research output: Contribution to journalReview article

Neurodegenerative Diseases
Proteins
Clinical Pathology
Mutant Proteins
polyglutamine
69 Citations (Scopus)

Polyglutamine spinocerebellar ataxias-from genes to potential treatments

Paulson, H. L., Shakkottai, V. G., Clark, H. B. & Orr, H. T., Sep 19 2017, In : Nature Reviews Neuroscience. 18, 10, p. 613-626 14 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Genes
Neurodegenerative Diseases
Glutamine
polyglutamine
8 Citations (Scopus)

Qs in the nucleus

Orr, H. T., Sep 27 2001, In : Neuron. 31, 6, p. 875-876 2 p.

Research output: Contribution to journalReview article

Retinal Degeneration
Poisons
Mutant Proteins
Neurodegenerative Diseases
Transcription Factors
58 Citations (Scopus)

Reversing neurodegeneration: A promise unfolds

Orr, H. T. & Zoghbi, H. Y., Mar 31 2000, In : Cell. 101, 1, p. 1-4 4 p.

Research output: Contribution to journalReview article

25 Citations (Scopus)

RNA targets of the fragile X Protein

Kaytor, M. D. & Orr, H. T., Nov 30 2001, In : Cell. 107, 5, p. 555-557 3 p.

Research output: Contribution to journalReview article

Fragile X Syndrome
Medical Genetics
Genomics
Drosophila
RNA
55 Citations (Scopus)

SCA1 molecular genetics: A history of a 13 year collaboration against glutamines

Orr, H. T. & Zoghbi, H. Y., Oct 1 2001, In : Human Molecular Genetics. 10, 20, p. 2307-2311 5 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Glutamine
Molecular Biology
Trinucleotide Repeat Expansion
Mutant Proteins
22 Citations (Scopus)

SCA1-Phosphorylation, a regulator of Ataxin-1 function and pathogenesis

Orr, H. T., Dec 1 2012, In : Progress in Neurobiology. 99, 3, p. 179-185 7 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Phosphorylation
Trinucleotide Repeats
Post Translational Protein Processing
Neurodegenerative Diseases

Spinocerebellar ataxia type 1

Lin, X., Zoghbi, H. Y. & Orr, H. T., Jan 1 2000, In : NeuroScience News. 3, 2-3, p. 81-86 6 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
24 Citations (Scopus)
Spinocerebellar Ataxias
Neurodegenerative Diseases
Transgenic Mice
Purkinje Cells
Mutant Proteins

Structural studies of the membrane-associated products of the human major histocompatibility complex.

Orr, H., Fuks, A., Kaufman, J., Lancet, D., Ploegh, H., Robb, R., Strominger, J. & Parham, P., Jan 1 1980, In : Advances in pathobiology. 7, p. 318-330 13 p.

Research output: Contribution to journalReview article

52 Citations (Scopus)

The genetics of neurodegenerative diseases

Hardy, J. & Orr, H., Jun 1 2006, In : Journal of Neurochemistry. 97, 6, p. 1690-1699 10 p.

Research output: Contribution to journalReview article

Neurodegenerative diseases
Inborn Genetic Diseases
Neurodegenerative Diseases
Nervous System Heredodegenerative Disorders
Motor Neuron Disease
172 Citations (Scopus)

The GSK3β signaling cascade and neurodegenerative disease

Kaytor, M. D. & Orr, H. T., Jun 1 2002, In : Current opinion in neurobiology. 12, 3, p. 275-278 4 p.

Research output: Contribution to journalReview article

Glycogen Synthase Kinase 3
Neurodegenerative Diseases
Neurofibrillary Tangles
Alzheimer Disease
Enzymes
17 Citations (Scopus)

The ins and outs of a polyglutamine neurodegenerative disease: Spinocerebellar ataxia type 1 (SCA1)

Orr, H. T., Jun 2000, In : Neurobiology of Disease. 7, 3, p. 129-134 6 p.

Research output: Contribution to journalReview article

Spinocerebellar Ataxias
Neurodegenerative Diseases
Mutant Proteins
Glutamine
Transgenic Mice
106 Citations (Scopus)

The unstable repeats-Three evolving faces of neurological disease

Nelson, D. L., Orr, H. T. & Warren, S. T., Jan 1 2013, In : Neuron. 77, 5, p. 825-843 19 p.

Research output: Contribution to journalReview article

Poisons
Neurosciences
Nervous System Diseases
Microsatellite Repeats
Nucleotides
922 Citations (Scopus)

Trinucleotide repeat disorders

Orr, H. T. & Zoghbi, H. Y., Aug 13 2007, In : Annual Review of Neuroscience. 30, p. 575-621 47 p.

Research output: Contribution to journalReview article

Trinucleotide Repeats
Protein Conformation
Neuronal Plasticity
Neurobiology
Muscular Dystrophies
Short survey
7 Citations (Scopus)

A proposed mechanism of ALS fails the test in vivo

Orr, H. T., Apr 16 2002, In : Nature neuroscience. 5, 4, p. 287-288 2 p.

Research output: Contribution to journalShort survey

Motor Neuron Disease
Poisons
Superoxide Dismutase
Free Radicals
2 Citations (Scopus)

Are Polyglutamine Diseases Expanding?

Orr, H. T., May 12 2011, In : Neuron. 70, 3, p. 377-378 2 p.

Research output: Contribution to journalShort survey

Antisense RNA
Peptides
Disease Progression
Neurons
polyglutamine
13 Citations (Scopus)

Fragile X Tremor/Ataxia Syndrome: Blame the Messenger!

Swanson, M. S. & Orr, H. T., Aug 16 2007, In : Neuron. 55, 4, p. 535-537 3 p.

Research output: Contribution to journalShort survey

RNA-Binding Proteins
Drosophila
Alleles
Neurons
Genes
18 Citations (Scopus)

FTD and ALS: Genetic ties that bind

Orr, H. T., Oct 20 2011, In : Neuron. 72, 2, p. 189-190 2 p.

Research output: Contribution to journalShort survey

Neurodegenerative Diseases
Mutation
Frontotemporal Dementia With Motor Neuron Disease
10 Citations (Scopus)

Neurodegenerative disease: Cut to the chase

Ellerby, L. M. & Orr, H. T., Aug 10 2006, In : Nature. 442, 7103, p. 641-642 2 p.

Research output: Contribution to journalShort survey

disease by anatomical location
27 Citations (Scopus)

Neuron protection agency

Orr, H. T., Oct 14 2004, In : Nature. 431, 7010, p. 747-748 2 p.

Research output: Contribution to journalShort survey

Nerve Tissue Proteins
Neostriatum
Biological Models
Huntington Disease
Inclusion Bodies
136 Citations (Scopus)

Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, Spinocerebellar ataxia type 1

Zoghbi, H. Y. & Orr, H. T., Mar 20 2009, In : Journal of Biological Chemistry. 284, 12, p. 7425-7429 5 p.

Research output: Contribution to journalShort survey

Neurodegenerative diseases
Spinocerebellar Ataxias
Neurodegenerative Diseases
Trinucleotide Repeats
Phosphorylation
6 Citations (Scopus)

RNA gains a new function: A mediator of neurodegeneration

Orr, H. T., May 1 2004, In : Trends in Neurosciences. 27, 5, p. 233-234 2 p.

Research output: Contribution to journalShort survey

RNA
Neurodegenerative Diseases
Tremor
Ataxia
Intellectual Disability
6 Citations (Scopus)
Nervous System Diseases
Nucleotides
Mutation