P. Thomas

发表

Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene

George P Patrinos, Rachel Karchin, David L. Masica, 2013, Nature Genetics.

The Molecular Chaperone Hsc70 Assists the in VitroFolding of the N-terminal Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator*

E. Strickland, L. Millen, P. Thomas, 1997, The Journal of Biological Chemistry.

Structure of nucleotide‐binding domain 1 of the cystic fibrosis transmembrane conductance regulator

J. M. Sauder, S. Burley, W. Hendrickson, 2004, The EMBO journal.

The Cystic Fibrosis-causing Mutation ΔF508 Affects Multiple Steps in Cystic Fibrosis Transmembrane Conductance Regulator Biogenesis*

H. Senderowitz, K. Du, G. Lukács, 2010, The Journal of Biological Chemistry.

CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices

J. Forman-Kay, V. Kanelis, W. Choy, 2007, Nature Structural &Molecular Biology.

Defective protein folding as a basis of human disease.

P. Pedersen, P. Thomas, B. Qu, 1995, Trends in biochemical sciences.

Organic Solutes Rescue the Functional Defect in ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator*

P. Thomas, S. Guggino, P. Thibodeau, 2003, Journal of Biological Chemistry.

Protein solubility and folding monitored in vivo by structural complementation of a genetic marker protein

John F. Hunt, Nathan M. Smith, Philip J. Thomas, 2001, Nature Biotechnology.

Regulation of Cl−/ HCO3 −Exchange by Cystic Fibrosis Transmembrane Conductance Regulator Expressed in NIH 3T3 and HEK 293 Cells*

M. G. Lee, Min Goo Lee, S. Muallem, 1999, The Journal of Biological Chemistry.

The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and ΔF508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein

P. Pedersen, P. Thomas, Michael. Delannoy, 1993 .

Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization

Yuh Min Chook, Y. Chook, P. Thomas, 2018, Scientific Reports.

A precipitating role for truncated alpha-synuclein and the proteasome in alpha-synuclein aggregation: implications for pathogenesis of Parkinson disease.

Karen A. Lewis, P. Thomas, B. Giasson, 2005, The Journal of biological chemistry.

Activity and Regulation of the Centrosome-associated Proteasome*

P. Thomas, G. Demartino, W. C. Wigley, 2000, The Journal of Biological Chemistry.

Alteration of CFTR transmembrane span integration by disease-causing mutations

L. Millen, P. Thomas, A. E. Patrick, 2011, Molecular biology of the cell.

Endoproteolytic Activity of the Proteasome

P. Thomas, G. Demartino, Chang-Wei Liu, 2002, Science.

Hook2 contributes to aggresome formation

H. Krämer, P. Thomas, W. C. Wigley, 2007, BMC Cell Biology.

Coupling Modes and Stoichiometry of Cl−/HCO3 − Exchange by slc26a3 and slc26a6

S. Muallem, P. Thomas, S. Ko, 2006, The Journal of general physiology.

Coupling Modes and Stoichiometry of Cl − / HCO 3 − Exchange by slc 26 a 3 and slc 26 a 6

S. Muallem, P. Thomas, S. Ko, 2006 .

Slc26a6 regulates CFTR activity in vivo to determine pancreatic duct HCO3− secretion: relevance to cystic fibrosis

S. Muallem, P. Thomas, C. Marino, 2006, The EMBO journal.

A molecular mechanism for aberrantCFTR‐dependent HCO3− transport in cystic fibrosis

Min Goo Lee, S. Muallem, P. Thomas, 2002, The EMBO journal.

IRBIT governs epithelial secretion in mice by antagonizing the WNK/SPAK kinase pathway.

K. Mikoshiba, I. So, S. Muallem, 2011, The Journal of clinical investigation.

Pathogenic Signal Sequence Mutations in Progranulin Disrupt SRP Interactions Required for mRNA Stability

P. Thomas, E. Tikhonova, Emile S. Pinarbasi, 2018, Cell reports.

A role for the ribosome-associated complex in activation of the IRE1 branch of UPR.

P. Thomas, Yi Liu, W. Skach, 2020, Cell reports.

17β-Estradiol Dysregulates Innate Immune Responses to Pseudomonas aeruginosa Respiratory Infection and Is Modulated by Estrogen Receptor Antagonism

J. Katzenellenbogen, S. Brody, D. Greenberg, 2017, Infection and Immunity.

Cystic Fibrosis Transmembrane Conductance Regulator Regulates Luminal Cl−/HCO3 −Exchange in Mouse Submandibular and Pancreatic Ducts*

Min Goo Lee, S. Muallem, E. Strickland, 1999, The Journal of Biological Chemistry.

Immuno and functional characterization of CFTR in submandibular and pancreatic acinar and duct cells.

M. G. Lee, S. Muallem, S. Freedman, 1997, The American journal of physiology.

Aberrant CFTR-dependent HCO-3 transport in mutations associated with cystic fibrosis

Min Goo Lee, S. Muallem, P. Thomas, 2001, Nature.

Dynamic control of cystic fibrosis transmembrane conductance regulator Cl(-)/HCO3(-) selectivity by external Cl(-).

S. Muallem, H. Goto, P. Thomas, 2004, The Journal of biological chemistry.

Mitochondrial ATP synthase: Progress toward understanding the relationship between its unique structure and its biological function

P. Pedersen, D. Garboczi, L. Amzel, 1995 .

In vitro CFTR folding assays.

P. Thomas, W. C. Wigley, Rhesa D. Stidham, 2002, Methods in molecular medicine.

CFTR Folding Consortium: methods available for studies of CFTR folding and correction.

G. Lukács, H. Pollard, J. Brodsky, 2011, Methods in molecular biology.

Gating of CFTR by the STAS domain of SLC26 transporters

S. Muallem, H. Goto, L. Millen, 2004, Nature Cell Biology.

The Cystic Fibrosis Transmembrane Conductance Regulator Interacts with and Regulates the Activity of the HCO 3 − Salvage Transporter Human Na+-HCO 3 − Cotransport Isoform 3*

Min Goo Lee, S. Muallem, P. Thomas, 2002, The Journal of Biological Chemistry.

Congenital Chloride-losing Diarrhea Causing Mutations in the STAS Domain Result in Misfolding and Mistrafficking of SLC26A3*

S. Muallem, J. Forman-Kay, P. Thomas, 2008, Journal of Biological Chemistry.

Interferon gamma regulates accumulation of the proteasome activator PA28 and immunoproteasomes at nuclear PML bodies.

P. Thomas, G. Demartino, W. C. Wigley, 2001, Journal of cell science.

Inefficient SRP Interaction with a Nascent Chain Triggers a mRNA Quality Control Pathway

G. Heijne, I. Nilsson, Qinghua Liu, 2014, Cell.

Uncoupling retro‐translocation and degradation in the ER‐associated degradation of a soluble protein

M. Latterich, J. Brodsky, P. Thomas, 2004, The EMBO journal.

A Unique Redox-sensing Sensor II Motif in TorsinA Plays a Critical Role in Nucleotide and Partner Binding*

L. Millen, P. Thomas, J. Mendoza, 2010, The Journal of Biological Chemistry.

The torsin-family AAA+ protein OOC-5 contains a critical disulfide adjacent to Sensor-II that couples redox state to nucleotide binding.

L. Rose, J. Wrabl, P. Thomas, 2008, Molecular biology of the cell.

Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells.

A. Sivachenko, P. Thomas, H. Bihler, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

CFTR modulator theratyping: Current status, gaps and future directions.

J. Wallenburg, J. Clancy, J. Nick, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

The Crystal Structure of the MJ0796 ATP-binding Cassette

Y. Yuan, J. Hunt, L. Millen, 2001, The Journal of Biological Chemistry.

Long-term culture and cloning of primary human bronchial basal cells that maintain multipotent differentiation capacity and CFTR channel function.

J. Shay, P. Thomas, R. Laranger, 2018, American journal of physiology. Lung cellular and molecular physiology.

TDP-43 Identified from a Genome Wide RNAi Screen for SOD1 Regulators

M. Roth, P. Thomas, Shuguang Wei, 2012, PloS one.

Nucleotide Binding Domain Interactions During the Mechanochemical Reaction Cycle of ATP-Binding Cassette Transporters

P. Thomas, Jonathan Moody, 2005, Journal of bioenergetics and biomembranes.

Dynamic Association of Proteasomal Machinery with the Centrosome

Min Goo Lee, S. Muallem, P. Thomas, 1999, The Journal of cell biology.

ATP binding to the motor domain from an ABC transporter drives formation of a nucleotide sandwich dimer.

John F Hunt, J. Hunt, N. Karpowich, 2002, Molecular cell.

Crystal structures of the MJ1267 ATP binding cassette reveal an induced-fit effect at the ATPase active site of an ABC transporter.

J. Hunt, N. Karpowich, L. Millen, 2001, Structure.

The cystic fibrosis transmembrane conductance regulator. Nucleotide binding to a synthetic peptide segment from the second predicted nucleotide binding fold.

P. Pedersen, P. Thomas, Y. Ko, 1994, The Journal of biological chemistry.

Side chain and backbone contributions of Phe508 to CFTR folding

Chad A Brautigam, M. Machius, P. Thomas, 2005, Nature Structural &Molecular Biology.

ATP binding and ATP hydrolysis play distinct roles in the function of 26S proteasome.

Hongtao Yu, P. Thomas, Xiaohua Li, 2006, Molecular cell.

Direct Binding of the Corrector VX-809 to Human CFTR NBD1: Evidence of an Allosteric Coupling between the Binding Site and the NBD1:CL4 Interface

Zhengrong Yang, Julie D Forman-Kay, J. Forman-Kay, 2017, Molecular Pharmacology.

Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences

Philip J. Thomas, Robert J. Bridges, André Schmidt, 2012, Cell.

The Primary Folding Defect and Rescue of ΔF508 CFTR Emerge during Translation of the Mutant Domain

Philip J. Thomas, P. Thomas, I. Braakman, 2010, PloS one.

Localization and Suppression of a Kinetic Defect in Cystic Fibrosis Transmembrane Conductance Regulator Folding*

E. Strickland, P. Thomas, B. Qu, 1997, The Journal of Biological Chemistry.

Alteration of the Cystic Fibrosis Transmembrane Conductance Regulator Folding Pathway

P. Thomas, B. Qu, 1996, The Journal of Biological Chemistry.

Cooperative, ATP-dependent Association of the Nucleotide Binding Cassettes during the Catalytic Cycle of ATP-binding Cassette Transporters*

John F Hunt, J. Hunt, L. Millen, 2002, The Journal of Biological Chemistry.

Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions

J. Forman-Kay, R. Muhandiram, P. Thomas, 2013, Proceedings of the National Academy of Sciences.

Conformational Remodeling of Proteasomal Substrates by PA700, the 19 S Regulatory Complex of the 26 S Proteasome*

L. Millen, P. Thomas, H. Gilbert, 2002, The Journal of Biological Chemistry.

Recognition of Misfolding Proteins by PA700, the Regulatory Subcomplex of the 26 S Proteasome*

E. Strickland, P. Thomas, G. Demartino, 2000, The Journal of Biological Chemistry.

CFTR degradation and aggregation.

P. Thomas, M. J. Corboy, W. Wigley, 2002, Methods in molecular medicine.

The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.

P. Pedersen, J. Sondek, P. Thomas, 1992, The Journal of biological chemistry.

A Precipitating Role for Truncated α-Synuclein and the Proteasome in α-Synuclein Aggregation

Philip J. Thomas, George N. DeMartino, Karen A. Lewis, 2005, Journal of Biological Chemistry.

Accelerated formation of α-synuclein oligomers by concerted action of the 20S proteasome and familial Parkinson mutations

Karen A. Lewis, P. Thomas, G. Demartino, 2010, Journal of bioenergetics and biomembranes.

Cystic fibrosis: recent structural insights.

P. Thomas, M. Dorwart, P. Thibodeau, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

A snapshot of Nature's favorite pump

J. Hunt, P. Thomas, 2001, Nature Structural Biology.

Characterization of novel small-molecule NRF2 activators: Structural and biochemical validation of stereospecific KEAP1 binding.

I. Dulubova, V. Stoll, K. Swinger, 2016, Biochimica et biophysica acta.

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.

John R Yates, Gerard Manning, Yoshihiro Matsumura, 2010, Nature chemical biology.

A Screen to Identify Cellular Modulators of Soluble Levels of an Amyotrophic Lateral Sclerosis (ALS)–Causing Mutant SOD1

P. Thomas, G. Miller, Balajee R. Somalinga, 2011, Journal of biomolecular screening.

Assessment of protein folding/solubility in live cells.

J. Hunt, P. Thomas, Rhesa D. Stidham, 2003, Methods in molecular biology.

Altered protein folding may be the molecular basis of most cases of cystic fibrosis

P. Pedersen, P. Thomas, Y. Ko, 1992, FEBS letters.

Chromosomal localization of genes required for the terminal steps of oxidative metabolism: α and γ subunits of ATP synthase and the phosphate carrier

E. Jabs, P. Pedersen, P. Thomas, 1994, Human Genetics.

Chromosomal localization of genes required for the terminal steps of oxidative metabolism: alpha and gamma subunits of ATP synthase and the phosphate carrier.

E. Jabs, P. Pedersen, P. Thomas, 1994, Human genetics.

Building an understanding of cystic fibrosis on the foundation of ABC transporter structures

P. Thomas, J. Mendoza, 2007, Journal of bioenergetics and biomembranes.

The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein.

P. Pedersen, P. Thomas, Y. Ko, 1993, Journal of Biological Chemistry.

Aggresome formation.

P. Thomas, M. J. Corboy, W. Wigley, 2005, Methods in molecular biology.

Biochemical and biophysical approaches to probe CFTR structure.

P. Thomas, J. Mendoza, A. Schmidt, 2011, Methods in molecular biology.

Folding Biology of Cystic Fibrosis: A Consortium‐Based Approach to Disease

G. Lukács, H. Pollard, J. Brodsky, 2010 .

A Conserved GXXXG Motif in APH-1 Is Critical for Assembly and Activity of the γ-Secretase Complex*

P. Thomas, Sheu-Fen Lee, J. Lundkvist, 2004, Journal of Biological Chemistry.

Recognition and processing of misfolded proteins by PA700, the 19S regulatory complex of the 26S proteasome.

E. Strickland, P. Thomas, G. Demartino, 2005, Methods in molecular biology.

The BiP Molecular Chaperone Plays Multiple Roles during the Biogenesis of TorsinA, an AAA+ ATPase Associated with the Neurological Disease Early-onset Torsion Dystonia*

J. Brodsky, M. Zółkiewski, L. Millen, 2014, The Journal of Biological Chemistry.

Abnormal neurites containing C-terminally truncated alpha-synuclein are present in Alzheimer's disease without conventional Lewy body pathology.

Karen A. Lewis, L. Hynan, P. Thomas, 2010, The American journal of pathology.

Cystic Fibrosis: A Disease of Altered Protein Folding

Philip J. Thomas, Elizabeth Strickland, E. Strickland, 1997, Journal of bioenergetics and biomembranes.

Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization

Y. Chook, P. Thomas, H. Fung, 2018, Scientific Reports.

Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation.

M. Amaral, P. Thomas, C. Farinha, 2008, Chemistry & biology.

NMR evidence for differential phosphorylation‐dependent interactions in WT and ΔF508 CFTR

Julie D Forman-Kay, J. Forman-Kay, V. Kanelis, 2010, The EMBO journal.

Mutations That Change the Position of the Putative γ-Phosphate Linker in the Nucleotide Binding Domains of CFTR Alter Channel Gating*

M. Welsh, J. Hunt, P. Thomas, 2002, The Journal of Biological Chemistry.

Alpha-synuclein truncation and disease

P. Thomas, C. Ritchie, 2012 .

Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening

Efrat Ben-Zeev, Martin Mense, Ori Kalid, 2010, J. Comput. Aided Mol. Des..

Development of CFTR Structure

Philip J. Thomas, P. Thomas, Anna E. Patrick, 2012, Front. Pharmacol..

Regulatory interaction between CFTR and the SLC26 transporters.

S. Muallem, P. Thomas, S. Ko, 2006, Novartis Foundation symposium.

F‐type ATPases: Are Nucleotide Domains in Adenylate Kinase Appropriate Models for Nucleotide Domains in ATP Synthase/ATPase Complexes? a

P. Pedersen, D. Garboczi, L. Amzel, 1992, Annals of the New York Academy of Sciences.

In Vitro Modulator Responsiveness of 655 CFTR Variants Found in People With CF

A. Sivachenko, G. Cutting, L. Millen, 2023, bioRxiv.

Role of transmembrane spanning domain 1 in cystic fibrosis transmembrane conductance regulator folding

L. Millen, P. Thomas, A. Patrick, 2021, bioRxiv.

Effects of the ΔF508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR

P. Pedersen, P. Thomas, 1993, Journal of bioenergetics and biomembranes.

A protein sequence that can encode native structure by disfavoring alternate conformations

Min Goo Lee, J. Rizo, J. Hunt, 2002, Nature Structural Biology.

The Cystic Fibrosis Transmembrane Conductance Regulator Interacts with and Regulates the Activity of the HCO 3 − Salvage Transporter Human Na+-HCO 3 − Cotransport Isoform 3*

Min Goo Lee, S. Muallem, P. Thomas, 2002, The Journal of Biological Chemistry.

Introduction to section IV: biophysical methods to approach CFTR structure.

P. Thomas, J. Mendoza, A. Schmidt, 2011, Methods in molecular biology.

Aberrant CFTR-dependent transport inmutations associated with cystic fibrosis

Min Goo Lee, S. Muallem, P. Thomas, 2014 .

N-Alpha-Acetyltransferases and Regulation of CFTR Expression

P. Thomas, A. Karamyshev, Henry Hudson, 2016, PloS one.

The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.

Joanne, J. Sondek, P. Thomas, 1992, The Journal of biological chemistry.

Hook2 contributes to aggresome formation

H. Krämer, P. Thomas, G. Szebenyi, 2007, BMC Cell Biology.

Direct Binding of the Corrector VX-809 to Human CFTR NBD1: Evidence of an Allosteric Coupling between the Binding Site and the NBD1:CL4 Interface

J. Forman-Kay, P. A. Chong, L. Millen, 2017, Molecular Pharmacology.

Nucleotide domains in transport ATPases: structure-function and relationship to disease.

P. Pedersen, P. Thomas, Y. Ko, 1995, Society of General Physiologists series.

A role for the ribosome-associated complex in activation of the IRE1 branch of UPR

P. Thomas, Yi Liu, W. Skach, 2021, Cell reports.