Publications by authors named "Bryce Sopher"

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Neuromuscular junction transcriptome analysis of spinal and bulbar muscular atrophy mice implicates sarcomere gene expression and calcium flux dysregulation in disease pathogenesis.
Anastasia Gromova, Byeonggu Cha, Nhat Nguyen, Diya Garg, Connor Coscolluela, Bryce L Sopher

Hum Mol Genet· July 2025

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Author Correction: Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy.
Nisha M Badders, Ane Korff, Helen C Miranda, Pradeep K Vuppala, Rebecca B Smith, Bryce L Sopher

Nat Med· March 2024

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Senataxin helicase, the causal gene defect in ALS4, is a significant modifier of C9orf72 ALS G4C2 and arginine-containing dipeptide repeat toxicity.
Craig L Bennett, Somasish Dastidar, Frederick J Arnold, Spencer U McKinstry, Cameron Stockford, Bryce L Sopher

Acta Neuropathol Commun· October 2023

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MAP4K3 inhibits Sirtuin-1 to repress the LKB1-AMPK pathway to promote amino acid-dependent activation of the mTORC1 complex.
Mary Rose Branch, Cynthia L Hsu, Kohta Ohnishi, Wen-Chuan Shen, Elian Lee, Bryce L Sopher

Life Sci Alliance· August 2023

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Altered H3 histone acetylation impairs high-fidelity DNA repair to promote cerebellar degeneration in spinocerebellar ataxia type 7.
Pawel M Switonski, Joe R Delaney, Luke C Bartelt, Chenchen Niu, Maria Ramos-Zapatero, Bryce L Sopher

Cell Rep· November 2021

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Early-Onset Familial Alzheimer Disease Variant PSEN2 N141I Heterozygosity is Associated with Altered Microglia Phenotype.
Susan Fung, Carole L Smith, Katherine E Prater, Amanda Case, Kevin Green, Bryce Sopher

J Alzheimers Dis· September 2021

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The pro-inflammatory microRNA miR-155 influences fibrillar β-Amyloid catabolism by microglia.
Macarena S Aloi, Katherine E Prater, Bryce Sopher, Stephanie Davidson, Suman Jayadev

Glia· July 2021

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4E-BP1 Protects Neurons from Misfolded Protein Stress and Parkinson's Disease Toxicity by Inducing the Mitochondrial Unfolded Protein Response.
Somasish Ghosh Dastidar, Michael T Pham, Matthew B Mitchell, Steven G Yeom, Sarah Jordan, Bryce L Sopher

J Neurosci· November 2020

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Tight expression regulation of senataxin, linked to motor neuron disease and ataxia, is required to avert cell-cycle block and nucleolus disassembly.
Craig L Bennett, Bryce L Sopher, Albert R La Spada

Heliyon· June 2020

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Inducible CRISPR genome editing platform in naive human embryonic stem cells reveals JARID2 function in self-renewal.
Amy Ferreccio, Julie Mathieu, Damien Detraux, Logeshwaran Somasundaram, Christopher Cavanaugh, Bryce Sopher

Cell Cycle· September 2019

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Alternative splicing in a presenilin 2 variant associated with Alzheimer disease.
Jacquelyn E Braggin, Stephanie A Bucks, Meredith M Course, Carole L Smith, Bryce Sopher

Ann Clin Transl Neurol· April 2019

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Neuronal susceptibility to beta-amyloid toxicity and ischemic injury involves histone deacetylase-2 regulation of endophilin-B1.
David B Wang, Chizuru Kinoshita, Yoshito Kinoshita, Bryce L Sopher, Takuma Uo

Brain Pathol· March 2019

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Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology.
H Jürgen Wenzel, Karl D Murray, Saif N Haify, Michael R Hunsaker, Jared J Schwartzer, Bryce L Sopher

Acta Neuropathol Commun· February 2019

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Metabolic and Organelle Morphology Defects in Mice and Human Patients Define Spinocerebellar Ataxia Type 7 as a Mitochondrial Disease.
Jacqueline M Ward, Colleen A Stoyas, Pawel M Switonski, Farid Ichou, Weiwei Fan, Bryce L Sopher

Cell Rep· January 2019

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Antisense oligonucleotides targeting mutant Ataxin-7 restore visual function in a mouse model of spinocerebellar ataxia type 7.
Chenchen Niu, Thazah P Prakash, Aneeza Kim, John L Quach, Laryssa A Huryn, Bryce L Sopher

Sci Transl Med· October 2018

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Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.
Craig L Bennett, Somasish G Dastidar, Shuo-Chien Ling, Bilal Malik, Travis Ashe, Bryce L Sopher

Acta Neuropathol· September 2018

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Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy.
Nisha M Badders, Ane Korff, Helen C Miranda, Pradeep K Vuppala, Rebecca B Smith, Bryce L Sopher

Nat Med· May 2018

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Recombinant adeno-associated viral (rAAV) vectors mediate efficient gene transduction in cultured neonatal and adult microglia.
Wei Su, John Kang, Bryce Sopher, James Gillespie, Macarena S Aloi

J Neurochem· January 2016

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PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically.
Audrey S Dickey, Victor V Pineda, Taiji Tsunemi, Patrick P Liu, Helen C Miranda, Bryce L Sopher

Nat Med· January 2016

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Proteolytic cleavage of ataxin-7 promotes SCA7 retinal degeneration and neurological dysfunction.
Stephan J Guyenet, Shona S Mookerjee, Amy Lin, Sara K Custer, Sylvia F Chen, Bryce L Sopher

Hum Mol Genet· July 2015

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Loss of endophilin-B1 exacerbates Alzheimer's disease pathology.
David B Wang, Yoshito Kinoshita, Chizuru Kinoshita, Takuma Uo, Bryce L Sopher

Brain· July 2015

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Let-7 coordinately suppresses components of the amino acid sensing pathway to repress mTORC1 and induce autophagy.
Amy N Dubinsky, Somasish Ghosh Dastidar, Cynthia L Hsu, Rabaab Zahra, Stevan N Djakovic, Bryce L Sopher

Cell Metab· October 2014

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Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA.
Constanza J Cortes, Helen C Miranda, Harald Frankowski, Yakup Batlevi, Jessica E Young, Bryce L Sopher

Nat Neurosci· September 2014

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Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy.
Constanza J Cortes, Shuo-Chien Ling, Ling T Guo, Gene Hung, Taiji Tsunemi, Bryce L Sopher

Neuron· April 2014

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Bax interacting factor-1 promotes survival and mitochondrial elongation in neurons.
David B Wang, Takuma Uo, Chizuru Kinoshita, Bryce L Sopher, Rona J Lee

J Neurosci· February 2014

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