Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency

Erin E. Cummings; Linda P. O’Reilly; Dale E. King; Richard M. Silverman; Mark T. Miedel; Cliff J. Luke; David H. Perlmutter; Gary A. Silverman; Stephen C. Pak

doi:10.1371/journal.pone.0141542

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Peer-reviewed

Research Article

Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency

Erin E. Cummings ,

Contributed equally to this work with: Erin E. Cummings, Linda P. O’Reilly

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯
Linda P. O’Reilly ,

Contributed equally to this work with: Erin E. Cummings, Linda P. O’Reilly

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯
Dale E. King,

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯
Richard M. Silverman,

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯
Mark T. Miedel,

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯
Cliff J. Luke,

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯
David H. Perlmutter,

Affiliations Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America, Department of Cell Biology and Molecular Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
⨯
Gary A. Silverman ,

* E-mail: paksc@upmc.edu (SCP); gsilverman@upmc.edu (GAS)

Affiliations Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America, Department of Cell Biology and Molecular Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
⨯
Stephen C. Pak

* E-mail: paksc@upmc.edu (SCP); gsilverman@upmc.edu (GAS)

Affiliation Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, United States of America
⨯

Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency

Erin E. Cummings,
Linda P. O’Reilly,
Dale E. King,
Richard M. Silverman,
Mark T. Miedel,
Cliff J. Luke,
David H. Perlmutter,
Gary A. Silverman,
Stephen C. Pak

Published: October 29, 2015
https://doi.org/10.1371/journal.pone.0141542

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