Harry T. Orr
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American professor of neuroscience
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(Suggest an Edit or Addition)Harry T. Orr's Published Works
Number of citations in a given year to any of this author's works
Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author
Published Works
- Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1 (1993) (1589)
- Trinucleotide repeat disorders. (2007) (1357)
- Glutamine repeats and neurodegeneration. (2000) (1268)
- Ataxin-1 Nuclear Localization and Aggregation Role in Polyglutamine-Induced Disease in SCA1 Transgenic Mice (1998) (1005)
- Genetic linkage evidence for a familial Alzheimer's disease locus on chromosome 14. (1992) (920)
- RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia (2004) (677)
- Identification of genes that modify ataxin-1-induced neurodegeneration (2000) (668)
- SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat (1995) (580)
- Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures (1997) (531)
- Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 (1998) (525)
- Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice (1999) (514)
- Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. (2001) (508)
- A human major histocompatibility complex class I gene that encodes a protein with a shortened cytoplasmic segment. (1987) (502)
- Evidence for a mechanism predisposing to intergenerational CAG repeat instability in spinocerebellar ataxia type I (1993) (476)
- Major histocompatibility antigens: The human (HLA-A,-B,-C) and murine (H-2K, H-2D) class I molecules (1981) (437)
- Interaction of Akt-Phosphorylated Ataxin-1 with 14-3-3 Mediates Neurodegeneration in Spinocerebellar Ataxia Type 1 (2003) (415)
- Genetic linkage studies suggest that Alzheimer's disease is not a single homogeneous disorder (1990) (413)
- Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1 (2000) (372)
- Identification and characterization of the gene causing type 1 spinocerebellar ataxia (1994) (349)
- Linkage map of the human major histocompatibility complex including the tumor necrosis factor genes. (1987) (336)
- Spinocerebellar ataxia type 5 in a family descended from the grandparents of President Lincoln maps to chromosome 11 (1994) (315)
- A Long CAG Repeat in the Mouse Sca1 Locus Replicates SCA1 Features and Reveals the Impact of Protein Solubility on Selective Neurodegeneration (2002) (314)
- Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1 (2008) (312)
- ATAXIN-1 Interacts with the Repressor Capicua in Its Native Complex to Cause SCA1 Neuropathology (2006) (307)
- Expression analysis of the ataxin–1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals (1995) (304)
- Serine 776 of Ataxin-1 Is Critical for Polyglutamine-Induced Disease in SCA1 Transgenic Mice (2003) (303)
- Linkage and mutational analysis of familial Alzheimer disease kindreds for the APP gene region. (1992) (271)
- Purkinje Cell Expression of a Mutant Allele of SCA1in Transgenic Mice Leads to Disparate Effects on Motor Behaviors, Followed by a Progressive Cerebellar Dysfunction and Histological Alterations (1997) (270)
- A gene responsible for cavernous malformations of the brain maps to chromosome 7q. (1995) (269)
- Recovery from Polyglutamine-Induced Neurodegeneration in Conditional SCA1 Transgenic Mice (2004) (261)
- The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1 (1997) (250)
- Transfer and expression of three cloned human non-HLA-A,B,C class I major histocompatibility complex genes in mutant lymphoblastoid cells. (1988) (246)
- HLA-E. A novel HLA class I gene expressed in resting T lymphocytes. (1988) (237)
- Cloning and complete sequence of an HLA-A2 gene: analysis of two HLA-A alleles at the nucleotide level. (1985) (228)
- Differential expression of HLA-E, HLA-F, and HLA-G transcripts in human tissue. (1990) (227)
- Assembly and maturation of HLA-A and HLA-B antigens in vivo (1979) (225)
- Polyglutamine spinocerebellar ataxias — from genes to potential treatments (2017) (222)
- Pathogenic Mechanisms of a Polyglutamine-mediated Neurodegenerative Disease, Spinocerebellar Ataxia Type 1* (2009) (215)
- The AXH Domain of Ataxin-1 Mediates Neurodegeneration through Its Interaction with Gfi-1/Senseless Proteins (2005) (212)
- Mice Lacking Ataxin-1 Display Learning Deficits and Decreased Hippocampal Paired-Pulse Facilitation (1998) (207)
- Disrupted cerebellar cortical development and progressive degeneration of Purkinje cells in SV40 T antigen transgenic mice (1992) (206)
- RORα-Mediated Purkinje Cell Development Determines Disease Severity in Adult SCA1 Mice (2006) (200)
- Human leukocyte antigen F (HLA-F). An expressed HLA gene composed of a class I coding sequence linked to a novel transcribed repetitive element (1990) (198)
- miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis (2008) (198)
- Evidence for a novel gene for familial febrile convulsions, FEB2, linked to chromosome 19p in an extended family from the Midwest. (1998) (187)
- Gametic and somatic tissue–specific heterogeneity of the expanded SCA1 CAG repeat in spinocerebellar ataxia type 1 (1995) (187)
- The GSK3β signaling cascade and neurodegenerative disease (2002) (185)
- The Unstable Repeats—Three Evolving Faces of Neurological Disease (2013) (177)
- Mutations that impair a posttranscriptional step in expression of HLA-A and -B antigens. (1985) (176)
- Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice. (2004) (172)
- Beyond the Qs in the polyglutamine diseases. (2001) (169)
- Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle. (2006) (168)
- HLA and maternal‐fetal recognition (1992) (166)
- Lithium Therapy Improves Neurological Function and Hippocampal Dendritic Arborization in a Spinocerebellar Ataxia Type 1 Mouse Model (2007) (159)
- Spinocerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase. (1996) (146)
- An unfolded protein (2001) (145)
- Spinocerebellar ataxia type 1 and Machado-Joseph disease: incidence of CAG expansions among adult-onset ataxia patients from 311 families with dominant, recessive, or sporadic ataxia. (1995) (144)
- The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract. (2001) (143)
- Cell biology of spinocerebellar ataxia (2012) (142)
- SCA1-like Disease in Mice Expressing Wild-Type Ataxin-1 with a Serine to Aspartic Acid Replacement at Residue 776 (2010) (141)
- Tolerance is established in polyclonal CD4+ T cells by distinct mechanisms, according to self-peptide expression patterns (2016) (139)
- Carbohydrate moiety of HLA antigens. Antigenic properties and amino acid sequences around the site of glycosylation. (1977) (130)
- The heavy chain of human histocompatibility antigen HLA-B7 contains an immunoglobulin-like region (1979) (128)
- Pumilio1 Haploinsufficiency Leads to SCA1-like Neurodegeneration by Increasing Wild-Type Ataxin1 Levels (2015) (127)
- Partial Loss of Ataxin-1 Function Contributes to Transcriptional Dysregulation in Spinocerebellar Ataxia Type 1 Pathogenesis (2010) (126)
- Exercise and Genetic Rescue of SCA1 via the Transcriptional Repressor Capicua (2011) (122)
- RAS–MAPK–MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1 (2013) (118)
- The HLA class I gene family includes at least six genes and twelve pseudogenes and gene fragments. (1992) (117)
- Polyglutamine diseases: protein cleavage and aggregation (1999) (111)
- RNA association and nucleocytoplasmic shuttling by ataxin-1 (2005) (109)
- Molecular cloning of a human histocompatibility antigen cDNA fragment. (1980) (108)
- Aminopyridines Correct Early Dysfunction and Delay Neurodegeneration in a Mouse Model of Spinocerebellar Ataxia Type 1 (2011) (107)
- Complete amino acid sequence of a papain-solubilized human histocompatibility antigen, HLA-B7. 2. Sequence determination and search for homologies. (1979) (104)
- Chromosomal organization of the human major histocompatibility complex class I gene family [published erratum appears in J Exp Med 1989 Apr 1;169(4):1517] (1989) (104)
- Disruption of the ATXN1–CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans (2017) (103)
- Increased trinucleotide repeat instability with advanced maternal age. (1997) (103)
- Biosynthesis and cell surface localization of nonglycosylated human histocompatibility antigens. (1981) (103)
- Report of the Second International Workshop on Human Chromosome 6. (1994) (102)
- The GSK3 beta signaling cascade and neurodegenerative disease. (2002) (101)
- Isolation of HLA locus-specific DNA probes from the 3'-untranslated region. (1984) (100)
- Nuclear localization of the spinocerebellar ataxia type 7 protein, ataxin-7. (1999) (96)
- SUMOylation of the Polyglutamine Repeat Protein, Ataxin-1, Is Dependent on a Functional Nuclear Localization Signal* (2005) (96)
- Altered trafficking of membrane proteins in purkinje cells of SCA1 transgenic mice. (2001) (95)
- Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus. (2000) (94)
- Comparison of amino acid sequences of two human histocompatibility antigens, HLA-A2 and HLA-B7: location of putative alloantigenic sites. (1979) (93)
- Maternal/fetal interactions: the role of the MHC class I molecule HLA-G. (1993) (92)
- ATXN1 protein family and CIC regulate extracellular matrix remodeling and lung alveolarization. (2011) (92)
- The apolipoprotein E/CI/CII gene cluster and late-onset Alzheimer disease. (1994) (90)
- Antisense oligonucleotide-mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles. (2018) (90)
- Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes (2007) (89)
- Localization of the autosomal dominant HLA-linked spinocerebellar ataxia (SCA1) locus, in two kindreds, within an 8-cM subregion of chromosome 6p. (1991) (89)
- Identification and characterization of an ataxin-1-interacting protein: A1Up, a ubiquitin-like nuclear protein. (2000) (86)
- Emerging pathogenic pathways in the spinocerebellar ataxias. (2009) (86)
- Refined localization of the cerebral cavernous malformation gene (CCM1) to a 4-cM interval of chromosome 7q contained in a well-defined YAC contig. (1995) (86)
- Cerebellar Transcriptome Profiles of ATXN1 Transgenic Mice Reveal SCA1 Disease Progression and Protection Pathways (2016) (83)
- The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7 (2008) (83)
- Structure of crossreactive human histocompatibility antigens HLA-A28 and HLA-A2: possible implications for the generation of HLA polymorphism. (1982) (82)
- Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1 (2015) (80)
- Reversing Neurodegeneration:A Promise Unfolds (2000) (79)
- Neuronal Atrophy Early in Degenerative Ataxia Is a Compensatory Mechanism to Regulate Membrane Excitability (2015) (79)
- Noninvasive Detection of Presymptomatic and Progressive Neurodegeneration in a Mouse Model of Spinocerebellar Ataxia Type 1 (2010) (78)
- In Vivo Viability of Postmitotic Purkinje Neurons Requires pRb Family Member Function (1995) (77)
- Mapmodulin/Leucine-rich Acidic Nuclear Protein Binds the Light Chain of Microtubule-associated Protein 1B and Modulates Neuritogenesis* (2003) (74)
- Polyglutamine neurodegeneration: expanded glutamines enhance native functions. (2012) (74)
- Use of HLA loss mutants to analyse the structure of the human major histocompatibility complex (1982) (72)
- Abnormalities in the Climbing Fiber-Purkinje Cell Circuitry Contribute to Neuronal Dysfunction in ATXN1[82Q] Mice (2011) (72)
- SCA1 molecular genetics: a history of a 13 year collaboration against glutamines. (2001) (71)
- ATXN1-CIC Complex Is the Primary Driver of Cerebellar Pathology in Spinocerebellar Ataxia Type 1 through a Gain-of-Function Mechanism (2018) (70)
- Extensive cryptic splicing upon loss of RBM17 and TDP43 in neurodegeneration models. (2016) (69)
- Progress in pathogenesis studies of spinocerebellar ataxia type 1. (1999) (69)
- Cloning and developmental expression analysis of the murine homolog of the spinocerebellar ataxia type 1 gene (Sca1). (1996) (67)
- Targeted Deletion of a Single Sca8 Ataxia Locus Allele in Mice Causes Abnormal Gait, Progressive Loss of Motor Coordination, and Purkinje Cell Dendritic Deficits (2006) (67)
- Prioritized research recommendations from the National Institute of Neurological Disorders and Stroke Parkinson's Disease 2014 conference (2014) (67)
- Identification of a self-association region within the SCA1 gene product, ataxin-1. (1997) (66)
- Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3ε haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration (2011) (66)
- HLA-AR, an inactivated antigen-presenting locus related to HLA-A. Implications for the evolution of the MHC. (1990) (65)
- Extraembryonic expression of the human MHC class I gene HLA-G in transgenic mice. Evidence for a positive regulatory region located 1 kilobase 5' to the start site of transcription. (1993) (65)
- Transfer of cloned human class I major histocompatibility complex genes into HLA mutant human lymphoblastoid cells (1986) (62)
- Chromosome 14 and late-onset familial Alzheimer disease (FAD). (1993) (61)
- Regional differences of somatic CAG repeat instability do not account for selective neuronal vulnerability in a knock-in mouse model of SCA1. (2003) (61)
- HLA-J, a second inactivated class I HLA gene related to HLA-G and HLA-A. Implications for the evolution of the HLA-A-related genes. (1992) (59)
- HLA non-A,B,C class I genes: Their structure and expression (1990) (59)
- Organization of the human class I major histocompatibility complex genes (1987) (58)
- Missense mutations in the chromosome 14 familial Alzheimer's disease presenilin 1 gene (1998) (57)
- Familial cerebral cavernous angioma: A gene localized to a 15‐cm interval on chromosome 7q (1996) (56)
- Erratum: Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures (1998) (55)
- Partial loss of Tip60 slows mid-stage neurodegeneration in a spinocerebellar ataxia type 1 (SCA1) mouse model (2011) (54)
- Spinocerebellar ataxia: localization of an autosomal dominant locus between two markers on human chromosome 6. (1987) (53)
- Spinocerebellar Ataxia Type 1—Modeling the Pathogenesis of a Polyglutamine Neurodegenerative Disorder in Transgenic Mice (2000) (52)
- Phosphorylation of ATXN1 at Ser776 in the cerebellum (2009) (52)
- Calcium dynamics and electrophysiological properties of cerebellar Purkinje cells in SCA1 transgenic mice. (2001) (52)
- 14-3-3 Binding to Ataxin-1(ATXN1) Regulates Its Dephosphorylation at Ser-776 and Transport to the Nucleus* (2011) (51)
- Class I-like HLA genes map telomeric to the HLA-A2 locus in human cells (1983) (51)
- Purkinje Cell Ataxin-1 Modulates Climbing Fiber Synaptic Input in Developing and Adult Mouse Cerebellum (2013) (50)
- Molecular analysis of the variant alloantigen HLA-B27d (HLA-B*2703) identifies a unique single amino acid substitution. (1988) (46)
- Patterns of CAG repeat instability in the central nervous system and periphery in Huntington’s disease and in spinocerebellar ataxia type 1 (2020) (45)
- RBM17 Interacts with U2SURP and CHERP to Regulate Expression and Splicing of RNA-Processing Proteins (2018) (43)
- The Effects of the Polyglutamine Repeat Protein Ataxin-1 on the UbL-UBA Protein A1Up* (2004) (43)
- Mapping and cloning of the critical region for the spinocerebellar ataxia type 1 gene (SCA1) in a yeast artificial chromosome contig spanning 1.2 Mb. (1993) (42)
- Generation and Characterization of LANP/pp32 Null Mice (2004) (41)
- The genetics of neurodegenerative diseases (2006) (40)
- Reduction of Purkinje Cell Pathology in SCA1 Transgenic Mice by p53 Deletion (2001) (37)
- SCA1—Phosphorylation, a regulator of Ataxin-1 function and pathogenesis (2012) (37)
- Diversity of class I HLA molecules: functional and evolutionary interactions with T cells. (1989) (35)
- A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1 (2015) (35)
- HLA Class I (1998) (33)
- Neurodegenerative disease: Neuron protection agency (2004) (32)
- In vivo monitoring of recovery from neurodegeneration in conditional transgenic SCA1 mice (2011) (32)
- RNA Targets of the Fragile X Protein (2001) (31)
- Complete amino acid sequence of a papain-solubilized human histocompatibility antigen HLA-B7. 1. Isolation and amino acid composition of fragments and of tryptic and chymotryptic peptides. (1979) (30)
- The Transcription Factor E2F-1 in SV40 T Antigen-Induced Cerebellar Purkinje Cell Degeneration (1998) (30)
- FTD and ALS: Genetic Ties that Bind (2011) (29)
- Polyclonal CD4+ T cell tolerance is established by distinct mechanisms, according to self-peptide expression patterns (2015) (29)
- A cell-based screen for modulators of ataxin-1 phosphorylation. (2005) (29)
- Mouse Models of Human CAG Repeat Disorders (1997) (28)
- HLA‐G Transgenic Mice: A Model for Studying Expression and Function at the Maternal/Fetal Interface (1995) (27)
- Susceptibility to Cell Death Induced by Mutant SV40 T-Antigen Correlates with Purkinje Neuron Functional Development (1997) (26)
- The gene for autosomal dominant spinocerebellar ataxia (SCA1) maps centromeric to D6S89 and shows no recombination, in nine large kindreds, with a dinucleotide repeat at the AM10 locus. (1993) (26)
- Polyglutamine Disease Toxicity Is Regulated by Nemo-like Kinase in Spinocerebellar Ataxia Type 1 (2013) (25)
- A physical linkage map of HLA-A, -G, -7.5p, and -F. (1991) (25)
- Assessing recovery from neurodegeneration in spinocerebellar ataxia 1: Comparison of in vivo magnetic resonance spectroscopy with motor testing, gene expression and histology (2015) (24)
- Cytotoxic T lymphocyte recognition of HLA-G in mice. (1997) (23)
- Amino acids in a region of ataxin-1 outside of the polyglutamine tract influence the course of disease in SCA1 transgenic mice (2002) (22)
- Cellular distribution of HLA-G mRNA in transgenic mouse placentas. (1993) (22)
- Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model (2018) (22)
- Neurodegenerative disease: Cut to the chase (2006) (22)
- Motor neuron degeneration correlates with respiratory dysfunction in SCA1 (2018) (22)
- Spinocerebellar ataxia type 1. (1995) (22)
- Linkage of an Alzheimer disease susceptibility locus to markers on human chromosome 21. (1991) (22)
- Protocol for genetic testing in Huntington disease: three years of experience in Minnesota. (1991) (21)
- Regulation of HLA class I transcription in T cells. (1989) (21)
- The Ins and Outs of a Polyglutamine Neurodegenerative Disease: Spinocerebellar Ataxia Type 1 (SCA1) (2000) (20)
- Spinocerebellar Ataxia Type 1: Molecular Mechanisms of Neurodegeneration and Preclinical Studies. (2018) (20)
- Dinucleotide repeat polymorphism at the D6S109 locus. (1991) (20)
- Molecular and endocrine characterization of a mutation involving a recombination between the steroid 21-hydroxylase functional gene and pseudogene (1991) (19)
- Identification of a novel phosphorylation site in ataxin-1. (2005) (18)
- Fragile X Tremor/Ataxia Syndrome: Blame the Messenger! (2007) (18)
- Comparison of multiple HLA-A alleles at the DNA level by using Southern blotting and HLA-A-specific probes. (1985) (17)
- Diminishing return for mechanistic therapeutics with neurodegenerative disease duration? (2016) (17)
- Characterization of the Zebrafish atxn1/axh Gene Family (2009) (16)
- Temporal and spatial expression of HLA-G messenger RNA in extraembryonic tissues of transgenic mice. (1995) (16)
- Diagnosis of classical steroid 21-hydroxylase deficiency using an HLA-B locus-specific DNA-probe. (1988) (16)
- Genetically engineered mouse models of the trinucleotide-repeat spinocerebellar ataxias (2012) (15)
- PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1 (2018) (15)
- Use of DNA probes from the 5' flanking region of the HLA-B gene to examine polymorphism at the HLA-B locus. (1986) (15)
- Overexpression of CREB reduces CRE-mediated transcription: behavioral and cellular analyses in transgenic mice (2004) (15)
- Targeting inhibitory cerebellar circuitry to alleviate behavioral deficits in a mouse model for studying idiopathic autism (2020) (15)
- HLA Class I Gene Family: Characterization of Genes Encoding Non-HLA-A,B,C Proteins (1989) (15)
- Microarrays and polyglutamine disorders: reports from the Hereditary Disease Array Group. (2002) (14)
- Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows Spinocerebellar ataxia type 1 phenotypes (2015) (14)
- Use of Southern blotting to analyze the size and restriction fragment polymorphism of HLA class I DNA in the human population (1983) (14)
- The c-fos gene and early-onset familial Alzheimer's disease (1993) (14)
- Altered Capicua expression drives regional Purkinje neuron vulnerability through ion channel gene dysregulation in spinocerebellar ataxia type 1 (2020) (14)
- Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin‐1 (2007) (14)
- Frequency of the delta Phe508 mutation and correlation with XV.2c/KM-19 haplotypes in an American population of cystic fibrosis patients: results of a collaborative study. (1990) (13)
- Pathogenesis of polyglutamine-induced disease: A model for SCA1. (1999) (13)
- Visualizing and mapping the cerebellum with serial optical coherence scanner (2016) (13)
- Autistic-like behavior and cerebellar dysfunction in Bmal1 mutant mice ameliorated by mTORC1 inhibition (2022) (13)
- Regulation of ataxin-1 phosphorylation and its impact on biology. (2013) (12)
- The Major Histocompatibility Complex: Analysis at the Protein and DNA Levels (1982) (12)
- Unstable trinucleotide repeats and the diagnosis of neurodegenerative disease. (1994) (11)
- A proposed mechanism of ALS fails the test in vivo (2002) (10)
- Lurcher, nPIST, and Autophagy (2002) (10)
- Into the depths of ataxia. (2004) (10)
- 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 (2008) (10)
- Toxic RNA as a driver of disease in a common form of ALS and dementia (2013) (9)
- Treadmill training increases the motor activity and neuron survival of the cerebellum in a mouse model of spinocerebellar ataxia type 1 (2019) (9)
- Autosomal dominant spinocerebellar ataxia (1992) (9)
- Unstable Nucleotide Repeat Minireview Series: A Molecular Biography of Unstable Repeat Disorders* (2008) (9)
- RNA gains a new function: a mediator of neurodegeneration (2004) (8)
- Consensus Paper: Strengths and Weaknesses of Animal Models of Spinocerebellar Ataxias and Their Clinical Implications (2021) (8)
- Generation and Characterization of LANP / pp 32 Null Mice (2004) (8)
- Antisense Oligonucleotide Therapeutic Approach for Suppression of Ataxin-1 Expression: A Safety Assessment (2020) (8)
- Modulation of ATXN1 S776 phosphorylation reveals the importance of allele-specific targeting in SCA1 (2021) (7)
- Qs in the Nucleus (2001) (7)
- Polarization-sensitive optical coherence tomography reveals gray matter and white matter atrophy in SCA1 mouse models (2018) (6)
- Mapping of class I DNA sequences within the human major histocompatibility complex (2004) (6)
- Cholecystokinin 1 receptor activation restores normal mTORC1 signaling and is protective to Purkinje cells of SCA mice (2021) (6)
- Structural aspects of the products of the human major histocompatibility complex. (1977) (6)
- Are Polyglutamine Diseases Expanding? (2011) (6)
- Dual targeting of brain region‐specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1 (2021) (5)
- Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1 (2021) (5)
- Toward understanding polyglutamine-induced neurological disease in spinocerebellar ataxia type 1. (1996) (5)
- Genetic approaches to pathogenesis of neurodegenerative diseases. (1995) (5)
- Linkage studies in dominantly inherited ataxias. (1993) (4)
- Correction: The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1 (1998) (4)
- Early stage of Spinocerebellar Ataxia Type 1 (SCA1) progression exhibits region- and cell-specific pathology and is partially ameliorated by Brain Derived Neurotrophic Factor (BDNF) (2021) (4)
- Reduction of mutant ATXN1 rescues premature death in a conditional SCA1 mouse model (2022) (3)
- Monoclonal Antibodies for Analysis of HLA Antigens: Further Studies with the W6/32 Antibody (1983) (3)
- The cell biology of disease Cell biology of spinocerebellar ataxia (2012) (3)
- Chapter 4 Complete Primary Structure of Human Histocompatibility Antigen Hla-B7: Evolutionary and Functional Implications (1980) (3)
- Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1 (2022) (3)
- A polymorphic DNA probe, p1-10-2, from chromosome 6. (1990) (2)
- Hereditary ataxia. An unfolded protein. (2001) (2)
- Disrupting ATXN1 Nuclear Localization in a Knock-in SCA1 Mouse Model Improves a Spectrum of SCA1-Like Phenotypes and their Brain Region Associated Transcriptomic Profiles (2021) (2)
- Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1 (2022) (2)
- Erratum: 14-3-3 binding to Ataxin-1 (ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus (Journal of Biological Chemistry (2011) 286 (34606-34616)) (2013) (1)
- Transfer and Expression of Human Non-A,B,C Class I Genes in Human HLA A,B,C Null Lymphoblastoid Cells (1989) (1)
- Synthetic activity of corneal endothelium. (1972) (1)
- Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1 (2023) (1)
- 717. RNAi Therapy for Dominant Neurodegenerative Diseases (2004) (1)
- Spinocerebellar Ataxia 1 (SCA1) (2003) (1)
- Complete Primary Structure of Human Histocompatibility Antigen HLA-B7 (1981) (1)
- Specific patterns of self-antigen expression determine the mechanisms by which polyclonal self-reactive CD4+ T cells are tolerized. (2016) (1)
- Nucleic Acid Probes in Diagnosis of Human Genetic Disease (1989) (1)
- Cholecystokinin Activation of Cholecystokinin 1 Receptors: a Purkinje Cell Neuroprotective Pathway. (2022) (1)
- CHROMOSOMAL ORGANIZATION OF THE HUMAN MAJOR HISTOCOMPATIBILITY COMPLEX CLASS I GENE FAMILY By BEVERLY H . KOLLER, DANIEL E . GERAGHTY, ROBERT DEMARS,* (2003) (1)
- Decreasing mutant ATXN1 nuclear localization improves a spectrum of SCA1-like phenotypes and brain region transcriptomic profiles (2022) (1)
- Molecular genetics of the human leukocyte antigen complex. (2020) (1)
- The ataxin-1 interactome reveals direct connection with multiple disrupted nuclear transport pathways (2020) (1)
- Cystic Fibrosis: Chromosome 7 DNA Genotyping (1988) (1)
- Imaging mouse cerebellum with serial optical coherence scanner (Conference Presentation) (2017) (0)
- Disruption of the ATXN1–CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans (2017) (0)
- Gene profiling links SCA1 pathophysiology to glutamate signaling in Purkinje cells of transgenic mice (2004) (0)
- Cholecystokinin 1 Receptor (Cck1R) Normalizes mTORC1 signaling and is Protective to Purkinje cells of SCA Mice (2021) (0)
- 13 – Spinocerebellar Ataxia Type 1 (2007) (0)
- PURKINJE CELL DEATH IN TRANSGENIC MICE EXPRESSING AN INTACT OR TRUNCATED FORM OF SV40 T ANTIGEN (1993) (0)
- Complete primary structure of human histocompatibility antigen HLA-B7: evolutionary and functional implications. (1980) (0)
- S3-02-05 Neuronal repair in conditional SCA1 mice (2004) (0)
- Serial optical coherence scanner for imaging neurodegenerative diseases (2018) (0)
- White matter atrophy in spinocerebellar ataxia type 1 mouse models revealed by serial optical coherence scanner (Conference Presentation) (2018) (0)
- Bidirectional control of airway responsiveness by endogenous cannabinoids. (2000) (0)
- CHAPTER K3 – Animal Models of Spinocerebellar Ataxia Type 1 (SCA1) (2005) (0)
- Author response: A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1 (2015) (0)
- Contents page + Editorial Board (2004) (0)
- Intrinsic Brain Signaling Pathways: Targets of Neuron Degeneration (2011) (0)
- Editorial Board (2007) (0)
- Thecerebellar leucine-rich acidic nuclear protein interactswithataxin-1 (0)
- Modeling polyglutamine pathogenesis in mice: SCA1 (2000) (0)
- Animal Models of Spinocerebellar Ataxia Type 1 (2015) (0)
- Parallel Neurochemical Alterations as measured by high field MRS in Patients and Mice with Spinocerebellar Ataxia Type 1 ( SCA 1 ) (2008) (0)
- Creating Coordination in the Cerebellum Catania, 2–4 October 2003 (2004) (0)
- PURKINJE CELL HYPERTROPHY, A POSSIBLE COMPENSATORY MECHANISM IN SCA1-TRANSGENIC MICE (1997) (0)
- Purkinje-Enriched snRNA-seq in SCA7 Cerebellum Reveals Zebrin Identity Loss as a Central Feature of Polyglutamine Ataxias (2023) (0)
- 14-3-3 binding to Ataxin-1 (ATXN1) regulates its dephosphorylation at Ser-776 and transport to the nucleus. (2013) (0)
- Author response: Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows Spinocerebellar ataxia type 1 phenotypes (2015) (0)
- A MUTATION IN THE pRB-BINDING REGION OF SV40 T-ANTIGEN (Tag) INHIBITS TARGETED PURKINJE CELL DEATH IN TRANSGENIC MICE BUT DOES NOT PREVENT DEVELOPMENT OF ATAXIA: 197 (1995) (0)
- Detection of Early Neurochemical Changes Related to Neurodegeneration in a Spinocerebellar Ataxia Type 1 (SCA1) Mouse Model by 1H MRS at 9.4 Tesla (2010) (0)
- A humanmajorhistocompatibili ty complex class Igenethat encodes aprotein withashortened cytoplasmic segment (1987) (0)
- CHARACTERIZATION OF TRANSGENIC MICE MADE ATAXIC BY EXPRESSION OF A MUTANT ALLELE FOR ATAXIN-1 THAT CONTAINS AN EXPANDED CAG REPEAT (1996) (0)
- Polyglutamine tract vs. protein context in SCA1 pathogenesis (2020) (0)
- Transgenic Mouse Models of CAG Trinucleotide Repeat Neurologic Diseases (1999) (0)
- Ataxin-1 with an expanded glutamine tract induces disease via alterations in nuclear function (1998) (0)
- Chapter 22 – Phosphorylation of Ataxin-1: A Link Between Basic Research and Clinical Application in Spinocerebellar Ataxia Type 1 (2006) (0)
- Expanding our Understanding of Polyglutamine Disease Through Transgenic Mice (2001) (0)
- Transgenic overexpression of wild-type CREB blocks endogenous CREB function (2000) (0)
- One Family's Search to Explain a Fatal Neurological Disorder (2013) (0)
- Editor's evaluation: A novel and accurate full-length HTT mouse model for Huntington’s disease (2021) (0)
- The molecular genetics of spinocerebellar degenerations (1997) (0)
- Decision letter: Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription (2018) (0)
- Cholecystokinin 1 Receptor (Cck1R) Activates mTORC1 Signaling and is Protective to Purkinje Cells in SCA Mice (2021) (0)
- Protocol for genetic testing in Huntington disease (1991) (0)
- The Impact of the Human Genome Project for Genetic Counseling Services (2020) (0)
- Decision letter: A novel and accurate full-length HTT mouse model for Huntington’s disease (2021) (0)
- Comparison of amino acid sequences of two human histocompatibility antigens , HLA-A 2 and HLA-B 7 : Location of putative alloantigenic sites ( protein structure / genetic polymorphism / alloantigenic determinants ) (0)
- Pathophysiology of SCA1 (2001) (0)
- Regional vulnerability in a neurodegenerative disease: Delineating SCA1 CNS and muscle therapeutic targets using a conditional mutant ATXN1 mouse (2023) (0)
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What Schools Are Affiliated With Harry T. Orr?
Harry T. Orr is affiliated with the following schools:
