Search Results - "Schlebach, Jonathan P."

Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis by Marinko, Justin T, Huang, Hui, Penn, Wesley D, Capra, John A, Schlebach, Jonathan P, Sanders, Charles R

“…Advances over the past 25 years have revealed much about how the structural properties of membranes and associated proteins are linked to the thermodynamics…”
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Benchmarking AlphaMissense pathogenicity predictions against cystic fibrosis variants by McDonald, Eli Fritz, Oliver, Kathryn E, Schlebach, Jonathan P, Meiler, Jens, Plate, Lars

“…Variants in the cystic fibrosis transmembrane conductance regulator gene (CFTR) result in cystic fibrosis-a lethal autosomal recessive disorder. Missense…”
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Coordination of -1 programmed ribosomal frameshifting by transcript and nascent chain features revealed by deep mutational scanning by Carmody, Patrick J, Zimmer, Matthew H, Kuntz, Charles P, Harrington, Haley R, Duckworth, Kate E, Penn, Wesley D, Mukhopadhyay, Suchetana, Miller, Thomas F, Schlebach, Jonathan P

“…Abstract Programmed ribosomal frameshifting (PRF) is a translational recoding mechanism that enables the synthesis of multiple polypeptides from a single…”
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Contribution of Cotranslational Folding Defects to Membrane Protein Homeostasis by Roushar, Francis J, Gruenhagen, Timothy C, Penn, Wesley D, Li, Bian, Meiler, Jens, Jastrzebska, Beata, Schlebach, Jonathan P

“…Membrane proteins are prone to misfolding and degradation within the cell, yet the nature of the conformational defects involved in this process remain poorly…”
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The safety dance: biophysics of membrane protein folding and misfolding in a cellular context by Schlebach, Jonathan P, Sanders, Charles R

“…Most biological processes require the production and degradation of proteins, a task that weighs heavily on the cell. Mutations that compromise the…”
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Conformational Stability and Pathogenic Misfolding of the Integral Membrane Protein PMP22 by Schlebach, Jonathan P, Narayan, Malathi, Alford, Catherine, Mittendorf, Kathleen F, Carter, Bruce D, Li, Jun, Sanders, Charles R

“…Despite broad biochemical relevance, our understanding of the physiochemical reactions that limit the assembly and cellular trafficking of integral membrane…”
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Divergent folding-mediated epistasis among unstable membrane protein variants by Chamness, Laura M, Kuntz, Charles P, McKee, Andrew G, Penn, Wesley D, Hemmerich, Christopher M, Rusch, Douglas B, Woods, Hope, Dyotima, Meiler, Jens, Schlebach, Jonathan P

“…Many membrane proteins are prone to misfolding, which compromises their functional expression at the plasma membrane. This is particularly true for the…”
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Influence of Pathogenic Mutations on the Energetics of Translocon-Mediated Bilayer Integration of Transmembrane Helices by Schlebach, Jonathan P., Sanders, Charles R.

“…Aberrant protein folding and assembly contribute to a number of diseases, and efforts to rationalize how pathogenic mutations cause this phenomenon represent…”
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Regulators of Viral Frameshifting: More Than RNA Influences Translation Events by Penn, Wesley D, Harrington, Haley R, Schlebach, Jonathan P, Mukhopadhyay, Suchetana

“…Programmed ribosomal frameshifting (PRF) is a conserved translational recoding mechanism found in all branches of life and viruses. In bacteria, archaea, and…”
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Peripheral myelin protein 22 alters membrane architecture by Mittendorf, Kathleen F, Marinko, Justin T, Hampton, Cheri M, Ke, Zunlong, Hadziselimovic, Arina, Schlebach, Jonathan P, Law, Cheryl L, Li, Jun, Wright, Elizabeth R, Sanders, Charles R, Ohi, Melanie D

“…Peripheral myelin protein 22 (PMP22) is highly expressed in myelinating Schwann cells of the peripheral nervous system. genetic alterations cause the most…”
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A protein folding intermediate pulls its weight by Schlebach, Jonathan P.

“…Proteins must acquire and maintain a specific fold to execute their biochemical function(s). In solution, unfolded proteins typically find this native…”
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Ions at the Interface: Pushing the pK of pHLIP by Schlebach, Jonathan P.

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Structure–Activity Relationship Study of Cannabidiol-Based Analogs as Negative Allosteric Modulators of the μ‑Opioid Receptor by Bosquez-Berger, Taryn, Gudorf, Jessica A., Kuntz, Charles P., Desmond, Jacob A., Schlebach, Jonathan P., VanNieuwenhze, Michael S., Straiker, Alex

“…The US faces an unprecedented surge in fatal drug overdoses. Naloxone, the only antidote for opiate overdose, competes at the mu opioid receptor (μOR)…”
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Glycosylation limits forward trafficking of the tetraspan membrane protein PMP22 by Marinko, Justin T., Wright, Madison T., Schlebach, Jonathan P., Clowes, Katherine R., Heintzman, Darren R., Plate, Lars, Sanders, Charles R.

“…Peripheral myelin protein 22 (PMP22) folds and trafficks inefficiently, with only 20% of newly expressed protein trafficking to the cell surface. This behavior…”
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Systematic profiling of temperature- and retinal-sensitive rhodopsin variants by deep mutational scanning by McKee, Andrew G., Kuntz, Charles P., Ortega, Joseph T., Woods, Hope, Most, Victoria, Roushar, Francis J., Meiler, Jens, Jastrzebska, Beata, Schlebach, Jonathan P.

“…Membrane protein variants with diminished conformational stability often exhibit enhanced cellular expression at reduced growth temperatures. The expression of…”
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Simplified proteomics approach to discover protein–ligand interactions by Chang, Youngil, Schlebach, Jonathan P., VerHeul, Ross A., Park, Chiwook

“…Identifying targets of biologically active small molecules is an essential but still challenging task in drug research and chemical genetics. Energetics‐based…”
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Chromenone derivatives as novel pharmacological chaperones for retinitis pigmentosa-linked rod opsin mutants by Ortega, Joseph T, McKee, Andrew G, Roushar, Francis J, Penn, Wesley D, Schlebach, Jonathan P, Jastrzebska, Beata

“…The correct expression of folded, functional rhodopsin (Rho) is critical for visual perception. However, this seven-transmembrane helical G protein-coupled…”
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General trends in the effects of VX-661 and VX-445 on the plasma membrane expression of clinical CFTR variants by McKee, Andrew G, McDonald, Eli F, Penn, Wesley D, Kuntz, Charles P, Noguera, Karen, Chamness, Laura M, Roushar, Francis J, Meiler, Jens, Oliver, Kathryn E, Plate, Lars, Schlebach, Jonathan P

“…Cystic fibrosis (CF) is caused by mutations that compromise the expression and/or function of the cystic fibrosis transmembrane conductance regulator (CFTR)…”
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Cotranslational folding stimulates programmed ribosomal frameshifting in the alphavirus structural polyprotein by Harrington, Haley R., Zimmer, Matthew H., Chamness, Laura M., Nash, Veronica, Penn, Wesley D., Miller, Thomas F., Mukhopadhyay, Suchetana, Schlebach, Jonathan P.

“…Viruses maximize their genetic coding capacity through a variety of biochemical mechanisms, including programmed ribosomal frameshifting (PRF), which…”
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Towards generalizable predictions for G protein-coupled receptor variant expression by Kuntz, Charles P., Woods, Hope, McKee, Andrew G., Zelt, Nathan B., Mendenhall, Jeffrey L., Meiler, Jens, Schlebach, Jonathan P.

“…Missense mutations that compromise the plasma membrane expression (PME) of integral membrane proteins are the root cause of numerous genetic diseases…”
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