Brandon Stauffer

发表

Potentiators exert distinct effects on human, murine, and Xenopus CFTR.

H. Senderowitz, N. Khazanov, Barry R. Imhoff, 2016, American journal of physiology. Lung cellular and molecular physiology.

Junctional abnormalities in human airway epithelial cells expressing F508del CFTR.

N. McCarty, Michael Koval, Samuel A. Molina, 2015, American journal of physiology. Lung cellular and molecular physiology.

An Ancient CFTR Ortholog Informs Molecular Evolution in ABC Transporters.

A. Gaggar, H. Senderowitz, J. Blalock, 2019, Developmental cell.

VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level

Barry R. Imhoff, N. McCarty, E. Sorscher, 2019, Scientific Reports.

Bacterial Sphingomyelinase is a State-Dependent Inhibitor of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR)

N. McCarty, B. Stauffer, K. Cottrill, 2017, Scientific Reports.

A mitochondrial long-chain fatty acid oxidation defect leads to uncharged tRNA accumulation and activation of the integrated stress response in the mouse heart

C. Argmann, C. Thompson, Aaron S. Bender, 2021, bioRxiv.

Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex

M. Arkin, Hui Wang, R. DeVita, 2020, bioRxiv.

A mitochondrial long-chain fatty acid oxidation defect leads to tRNA uncharging and activation of the integrated stress response in the mouse heart.

C. Argmann, Aaron S. Bender, J. Ellis, 2022, Cardiovascular research.

Deletion of 2‐aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1

R. DeVita, S. Houten, J. Leandro, 2020, Journal of inherited metabolic disease.

Inflammation and ER Stress Downregulate BDH2 Expression and Dysregulate Intracellular Iron in Macrophages

N. McCarty, S. Zughaier, B. Stauffer, 2014, Journal of immunology research.

The peroxisomal transporter ABCD3 plays a major role in hepatic dicarboxylic fatty acid metabolism and lipid homeostasis

S. Friedman, S. Houten, M. Puchowicz, 2021, Journal of inherited metabolic disease.

The peroxisomal transporter ABCD3 plays a major role in dicarboxylic fatty acid metabolism

S. Friedman, S. Houten, M. Puchowicz, 2021, bioRxiv.

Acyl‐CoA dehydrogenase substrate promiscuity: Challenges and opportunities for development of substrate reduction therapy in disorders of valine and isoleucine metabolism

R. DeVita, F. Vaz, S. Houten, 2023, Journal of inherited metabolic disease.

Acyl-CoA dehydrogenase substrate promiscuity limits the potential for development of substrate reduction therapy in disorders of valine and isoleucine metabolism

R. DeVita, S. Houten, J. Leandro, 2022, bioRxiv.