Publications by authors named "Laura C Bott"

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Nuclear receptor signaling via NHR-49/MDT-15 regulates stress resilience and proteostasis in response to reproductive and metabolic cues.
Ambre J Sala, Rogan A Grant, Ghania Imran, Claire Morton, Renee M Brielmann, Laura C Bott

Genes Dev· June 2024

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Hetero-oligomerization of TDP-43 carboxy-terminal fragments with cellular proteins contributes to proteotoxicity.
Akira Kitamura, Ai Fujimoto, Rei Kawashima, Yidan Lyu, Kotetsu Sasaki, Laura C Bott

Commun Biol· June 2024

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Nuclear receptor signaling via NHR-49/MDT-15 regulates stress resilience and proteostasis in response to reproductive and metabolic cues.
Ambre J Sala, Rogan A Grant, Ghania Imran, Claire Morton, Renee M Brielmann, Laura C Bott

bioRxiv· April 2023

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Variants in cause progressive myoclonus epilepsy and developmental and epileptic encephalopathy.
Laura C Bott, Mitra Forouhan, Maria Lieto, Ambre J Sala, Ruth Ellerington

Brain Commun· October 2021

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Embryo integrity regulates maternal proteostasis and stress resilience.
Ambre J Sala, Laura C Bott, Renee M Brielmann, Richard I Morimoto

Genes Dev· May 2020

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Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents.
Naemeh Pourshafie, Philip R Lee, Ke-Lian Chen, George G Harmison, Laura C Bott

J Vis Exp· August 2018

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Shaping proteostasis at the cellular, tissue, and organismal level.
Ambre J Sala, Laura C Bott, Richard I Morimoto

J Cell Biol· May 2017

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The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex.
Laura C Bott, Florian A Salomons, Dragan Maric, Yuhong Liu, Diane Merry

Sci Rep· June 2016

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MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy.
Naemeh Pourshafie, Philip R Lee, Ke-Lian Chen, George G Harmison, Laura C Bott

Mol Ther· May 2016

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A small-molecule Nrf1 and Nrf2 activator mitigates polyglutamine toxicity in spinal and bulbar muscular atrophy.
Laura C Bott, Nisha M Badders, Ke-Lian Chen, George G Harmison, Elaine Bautista

Hum Mol Genet· May 2016

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Sexual Reassignment Fails to Prevent Kennedy's Disease.
Tyler A Lanman, Dara Bakar, Nisha M Badders, Ailbhe Burke, Angela Kokkinis, Laura C Bott

J Neuromuscul Dis· March 2016

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The polyglutamine-expanded androgen receptor has increased DNA binding and reduced transcriptional activity.
Sergey Belikov, Laura C Bott, Kenneth H Fischbeck, Örjan Wrange

Biochem Biophys Rep· September 2015

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Mutation in CPT1C Associated With Pure Autosomal Dominant Spastic Paraplegia.
Carlo Rinaldi, Thomas Schmidt, Alan J Situ, Janel O Johnson, Philip R Lee, Laura C Bott

JAMA Neurol· May 2015

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Early onset and novel features in a spinal and bulbar muscular atrophy patient with a 68 CAG repeat.
Christopher Grunseich, Ilona R Kats, Laura C Bott, Carlo Rinaldi, Angela Kokkinis

Neuromuscul Disord· November 2014

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Stem cell-derived motor neurons from spinal and bulbar muscular atrophy patients.
Christopher Grunseich, Kristen Zukosky, Ilona R Kats, Laboni Ghosh, George G Harmison, Laura C Bott

Neurobiol Dis· October 2014

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The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution.
Nico P Dantuma, Laura C Bott

Front Mol Neurosci· August 2014

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Muscle matters in Kennedy's disease.
Carlo Rinaldi, Laura C Bott, Kenneth H Fischbeck

Neuron· April 2014

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Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy.
Carlo Rinaldi, Laura C Bott, Ke-lian Chen, George G Harmison, Masahisa Katsuno

Mol Med· December 2012

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VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD.
Emilie Tresse, Florian A Salomons, Jouni Vesa, Laura C Bott, Virginia Kimonis

Autophagy· February 2010

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A conserved unfoldase activity for the p97 AAA-ATPase in proteasomal degradation.
Anne Beskow, Kristian Björk Grimberg, Laura C Bott, Florian A Salomons, Nico P Dantuma

J Mol Biol· December 2009

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