Publications by authors named "Brandon Stauffer"

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Odd-Chain Dicarboxylic Acid Feeding Produces a Glutaric Aciduria Type 1-Like Metabolic Signature in Mice.
Adam C Richert, Yuxun Zhang, Sivakama S Bharathi, Abigail Hernandez, Tetyana Dodatko, Brandon Stauffer

FASEB J· November 2025

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Odd-chain dicarboxylic acid feeding recapitulates the biochemical phenotype of glutaric aciduria type 1 in mice.
Adam C Richert, Yuxun Zhang, Sivakama S Bharathi, Abigail Hernandez, Tetyana Dodatko, Brandon Stauffer

bioRxiv· February 2025

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Acyl-CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism.
Sander M Houten, Tetyana Dodatko, William Dwyer, Sara Violante, Hongjie Chen, Brandon Stauffer

J Inherit Metab Dis· September 2023

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A mitochondrial long-chain fatty acid oxidation defect leads to transfer RNA uncharging and activation of the integrated stress response in the mouse heart.
Pablo Ranea-Robles, Natalya N Pavlova, Aaron Bender, Andrea S Pereyra, Jessica M Ellis, Brandon Stauffer

Cardiovasc Res· December 2022

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Plasma Lysosphingolipid Biomarker Measurement by Liquid Chromatography Tandem Mass Spectrometry.
Brandon B Stauffer, Chunli Yu

Methods Mol Biol· September 2022

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The peroxisomal transporter ABCD3 plays a major role in hepatic dicarboxylic fatty acid metabolism and lipid homeostasis.
Pablo Ranea-Robles, Hongjie Chen, Brandon Stauffer, Chunli Yu, Dipankar Bhattacharya

J Inherit Metab Dis· November 2021

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Deletion of 2-aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1.
João Leandro, Tetyana Dodatko, Robert J DeVita, Hongjie Chen, Brandon Stauffer

J Inherit Metab Dis· November 2020

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Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex.
João Leandro, Susmita Khamrui, Hui Wang, Chalada Suebsuwong, Natalia S Nemeria, Brandon Stauffer

ACS Chem Biol· August 2020

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An Ancient CFTR Ortholog Informs Molecular Evolution in ABC Transporters.
Guiying Cui, Jeong Hong, Yu-Wen Chung-Davidson, Daniel Infield, Xin Xu, Brandon Stauffer

Dev Cell· November 2019

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VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
Guiying Cui, Brandon B Stauffer, Barry R Imhoff, Andras Rab, Jeong S Hong

Sci Rep· September 2019

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Potentiators exert distinct effects on human, murine, and Xenopus CFTR.
Guiying Cui, Netaly Khazanov, Brandon B Stauffer, Daniel T Infield, Barry R Imhoff

Am J Physiol Lung Cell Mol Physiol· August 2016

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Inflammation and ER stress downregulate BDH2 expression and dysregulate intracellular iron in macrophages.
Susu M Zughaier, Brandon B Stauffer, Nael A McCarty

J Immunol Res· December 2015

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Junctional abnormalities in human airway epithelial cells expressing F508del CFTR.
Samuel A Molina, Brandon Stauffer, Hannah K Moriarty, Agnes H Kim, Nael A McCarty

Am J Physiol Lung Cell Mol Physiol· September 2015

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CRIP1a switches cannabinoid receptor agonist/antagonist-mediated protection from glutamate excitotoxicity.
Brandon Stauffer, Kathleen T Wallis, Steven P Wilson, Michaela Egertová, Maurice R Elphick

Neurosci Lett· October 2011

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