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Research Output 1972 2018

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Review article
2017
50 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
2013
101 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
2012
98 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
11 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
50 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
20 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
2010
6 Citations (Scopus)

Nuclear ataxias.

Orr, H. T., May 1 2010, In : Cold Spring Harbor perspectives in biology. 2, 5

Research output: Contribution to journalReview article

Ataxia
Cytology
Neurodegenerative diseases
Neurosciences
Cell Nucleus
2009
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
2007
900 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
2006
116 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
50 Citations (Scopus)

The genetics of neurodegenerative diseases

Hardy, J. & Orr, H. T., 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
2004
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
2002
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
168 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
2001
141 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
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
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
54 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
2000
994 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
57 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

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
16 Citations (Scopus)

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

Orr, H. T., Jan 1 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
1999
88 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
1995
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
1993
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
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
1989

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
1981
339 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., Jan 1 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
1980

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