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Publications

19
Ribosomal Frameshifting Selectively Modulates the Biosynthesis, Assembly, and Function of a Misfolded CFTR Variant

Ribosomal Frameshifting Selectively Modulates the Biosynthesis, Assembly, and Function of a Misfolded CFTR Variant

Patrick Carmody, Francis J. Roushar, Austin Tedman, Madeline Herwig, Minsoo Kim, Eli F. McDonald, Jennifer Wong-Roushar, Jon-Luc Poirier, Nathan B. Zelt, Ben T. Pockrass, Andrew G. McKee, Charles P. Kuntz, Lars Plate, Wesley D. Penn, and Jonathan P. Schlebach. (2023) bioRxiv. https://www.biorxiv.org/content/10.1101/2023.05.02.539166v2

18
General Trends in the Effects of VX-661 and VX-445 on the Plasma Membrane Expression of Clinical CFTR Variants

General Trends in the Effects of VX-661 and VX-445 on the Plasma Membrane Expression of Clinical CFTR Variants

Andrew G McKee, Eli Fritz McDonald, Wesley D Penn, Charles P Kuntz, Karen Noguera, Laura M Chamness, Francis J Roushar,  Jens Meiler, Kathryn E Oliver, Lars Plate, and Jonathan P Schlebach (2023) Cell Chemical Biology. In Press

17
Chromenone derivatives as novel pharmacological chaperones for retinitis pigmentosa-linked rod opsin mutants

Chromenone derivatives as novel pharmacological chaperones for retinitis pigmentosa-linked rod opsin mutants

Joseph T Ortega, Andrew G McKee, Francis J Roushar, Wesley D Penn, Jonathan P Schlebach, and Beata Jastrzebska (2022) Human Molecular Genetics. 31, 3439-3457. https://academic.oup.com/hmg/advance-article/doi/10.1093/hmg/ddac125/6596553?login=true

16
Molecular Basis for Variations in the Sensitivity of Pathogenic Rhodopsin Variants to 9-cis-Retinal

Molecular Basis for Variations in the Sensitivity of Pathogenic Rhodopsin Variants to 9-cis-Retinal

Francis J. Roushar, Andrew G. McKee, Charles P. Kuntz, Joseph T. Ortega, Wesley D. Penn, Hope Woods, Laura M. Chamness, Victoria Most, Jens Meiler, Beata Jastrzebska, and Jonathan P. Schlebach (2022) Journal of Biological Chemistry. 298, 8. https://www.sciencedirect.com/science/article/pii/S0021925822007086?via%3Dihub

15
Towards Generalizable Predictions for the Effects of Mutations on G-Protein Coupled Receptor Expression

Towards Generalizable Predictions for the Effects of Mutations on G-Protein Coupled Receptor Expression

Charles P. Kuntz, Hope Woods, Andrew G. McKee, Nathan B. Zelt, Jeffrey L. Mendenhall, Jens Meiler, and Jonathan P. Schlebach (2022) Biophysical Journal 121, 2712-2720. https://www.sciencedirect.com/science/article/pii/S0006349522004775?via%3Dihub

14
Coordination of -1 programmed ribosomal frameshifting by transcript and nascent chain features revealed by deep mutational scanning

Coordination of -1 programmed ribosomal frameshifting by transcript and nascent chain features revealed by deep mutational scanning

Patrick J Carmody, Matthew H Zimmer, Charles P Kuntz, Haley R Harrington, Kate E Duckworth, Wesley D Penn, Suchetana Mukhopadhyay, Thomas F Miller 3rd, and Jonathan P Schlebach (2021) Nucleic Acids Research. 49, 12943-12954. https://academic.oup.com/nar/article/49/22/12943/6454274

13
Molecular basis for the evolved instability of a human G-protein coupled receptor

Molecular basis for the evolved instability of a human G-protein coupled receptor

Laura M Chamness, Nathan B Zelt, Haley R Harrington, Charles P Kuntz, Brian J Bender, Wesley D Penn, Joshua J Ziarek, Jens Meiler, and Jonathan P Schlebach (2021) Cell Reports. 37, 110046. https://www.cell.com/cell-reports/fulltext/S2211-1247(21)01532-1?_returnURL=https%3A%2F%2Flinkinghub.elsevier.com%2Fretrieve%2Fpii%2FS2211124721015321%3Fshowall%3Dtrue

12
Systematic profiling of temperature- and retinal-sensitive rhodopsin variants by deep mutational scanning

Systematic profiling of temperature- and retinal-sensitive rhodopsin variants by deep mutational scanning

Andrew G McKee, Charles P Kuntz, Joseph T Ortega, Hope Woods, Victoria Most, Francis J Roushar, Jens Meiler, Beata Jastrzebska, and Jonathan P Schlebach (2021) Journal of Biological Chemistry. 297, 101359.  

11
Disease-linked supertrafficking of a potassium channel

Disease-linked supertrafficking of a potassium channel

Hui Huang, Laura M Chamness, Carlos G Vanoye, Georg Kuenze, Jens Meiler, Alfred L George Jr, Jonathan P Schlebach, and Charles R Sanders (2021) Journal of Biological Chemistry. 296, 100423. https://www.jbc.org/article/S0021-9258(21)00196-4/fulltext

10
A protein folding intermediate pulls its weight

A protein folding intermediate pulls its weight

Jonathan P Schlebach (2020) Journal of Biological Chemistry. 295, 11418-11419. https://www.jbc.org/article/S0021-9258(17)48447-X/fulltext

9
Regulators of Viral Frameshifting: More Than RNA Influences Translation Events

Regulators of Viral Frameshifting: More Than RNA Influences Translation Events

Wesley D Penn, Haley R Harrington, Jonathan P Schlebach, and Suchetana Mukhopadhyay (2020) Annual Review of Virology. 7, 219-238. https://www.annualreviews.org/doi/10.1146/annurev-virology-012120-101548?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub++0pubmed

8
Classification of the Molecular Defects Associated with Pathogenic Variants of the SLC6A8 Creatine Transporter

Classification of the Molecular Defects Associated with Pathogenic Variants of the SLC6A8 Creatine Transporter

Martin D Salazar, Nathan B Zelt, Robert Saldivar, Charles P Kuntz, Sheng Chen, Wesley D Penn, Richard Bonneau, Julia Koehler Leman, and Jonathan P Schlebach (2020) Biochemistry. 59, 1367-1377. https://pubs.acs.org/doi/10.1021/acs.biochem.9b00956

7
Probing biophysical sequence constraints within the transmembrane domains of rhodopsin by deep mutational scanning

Probing biophysical sequence constraints within the transmembrane domains of rhodopsin by deep mutational scanning

Wesley D Penn, Andrew G McKee, Charles P Kuntz, Hope Woods, Veronica Nash, Timothy C Gruenhagen, Francis J Roushar, Mahesh Chandak, Chris Hemmerich, Douglas B Rusch, Jens Meiler, and Jonathan P Schlebach (2020) Science Advances 6, eaay7505. https://www.science.org/doi/10.1126/sciadv.aay7505?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed

6
Cotranslational folding stimulates programmed ribosomal frameshifting in the alphavirus structural polyprotein

Cotranslational folding stimulates programmed ribosomal frameshifting in the alphavirus structural polyprotein

Haley R Harrington, Matthew H Zimmer, Laura M Chamness, Veronica Nash, Wesley D Penn, Thomas F Miller 3rd, Suchetana Mukhopadhyay, and Jonathan P Schlebach (2020) Journal of Biological Chemistry. 295, 6798-6808. https://www.jbc.org/article/S0021-9258(17)48187-7/fulltext

5
Ions at the Interface: Pushing the pK of pHLIP

Ions at the Interface: Pushing the pK of pHLIP

Jonathan P Schlebach (2019) Biophysical Journal. 117, 793-794. https://www.cell.com/biophysj/fulltext/S0006-3495(19)30624-1

4
Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis

Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis

Justin T Marinko, Hui Huang, Wesley D Penn, John A Capra, Jonathan P Schlebach, and Charles R Sanders (2019) Chemical Reviews. 119, 5537-5606. https://pubs.acs.org/doi/10.1021/acs.chemrev.8b00532

3
Contribution of Cotranslational Folding Defects to Membrane Protein Homeostasis

Contribution of Cotranslational Folding Defects to Membrane Protein Homeostasis

Francis J Roushar, Timothy C Gruenhagen, Wesley D Penn, Bian Li, Jens Meiler, Beata Jastrzebska, and Jonathan P Schlebach (2019) Journal of the American Chemistry Society. 141, 204-215. https://pubs.acs.org/doi/10.1021/jacs.8b08243

2
The Retinitis Pigmentosa-Linked Mutations in Transmembrane Helix 5 of Rhodopsin Disrupt Cellular Trafficking Regardless of Oligomerization State

The Retinitis Pigmentosa-Linked Mutations in Transmembrane Helix 5 of Rhodopsin Disrupt Cellular Trafficking Regardless of Oligomerization State

D Paul Mallory, Elizabeth Gutierrez, Margaret Pinkevitch, Christie Klinginsmith, William D Comar, Francis J Roushar, Jonathan P Schlebach, Adam W Smith, and Beata Jastrzebska (2018) Biochemistry. 57, 5188-5201. https://pubs.acs.org/doi/10.1021/acs.biochem.8b00403

1
Bicelle size modulates the rate of bacteriorhodopsin folding

Bicelle size modulates the rate of bacteriorhodopsin folding

Timothy C Gruenhagen, Joshua J Ziarek, and  Jonathan P Schlebach (2018) Protein Science. 27, 1109-1112. https://onlinelibrary.wiley.com/doi/10.1002/pro.3414