Physiology - Division T

Research output

Research output per year 1990 1992 1997 2001 2002 2004 2005 2006 2010 2011 2019

• 86 Article
• 7 Conference contribution
• 3 Short survey
• 3 Review article
• 3 More
  • 2 Chapter
  • 1 Editorial

Research output per year

Research output per year

Search results

• 2019

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

  Valley, H. C., Bukis, K. M., Bell, A., Cheng, Y., Wong, E., Jordan, N. J., Allaire, N. E., Sivachenko, A., Liang, F., Bihler, H., Thomas, P. J., Mahiou, J. & Mense, M., Jul 2019, In: Journal of Cystic Fibrosis. 18, 4, p. 476-483 8 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Cystic Fibrosis 77%
  • Nonsense Mediated mRNA Decay 59%
  • Epithelial Cells 56%
  • Gene Editing 48%
  75 Scopus citations
• 2018

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

  Pinarbasi, E. S., Caǧatay, T., Fung, H. Y. J., Li, Y. C., Chook, Y. M. & Thomas, P. J., Dec 1 2018, In: Scientific reports. 8, 1, 7083.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Nuclear Export Signals 100%
  • Cell Nucleus Active Transport 78%
  • Nuclear Fusion 28%
  • Karyopherins 24%
  • RNA-Binding Proteins 19%
  83 Scopus citations

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

  Peters-Hall, J. R., Coquelin, M. L., Torres, M. J., Laranger, R., Alabi, B. R., Sho, S., Calva-Moreno, J. F., Thomas, P. J. & Shay, J. W., Aug 2018, In: American Journal of Physiology - Lung Cellular and Molecular Physiology. 315, 2, p. L313-L327

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Cellular Reprogramming 100%
  • Organism Cloning 95%
  • Epithelial Cells 74%
  • Gene Editing 47%
  • Cystic Fibrosis 25%
  42 Scopus citations

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

  Pinarbasi, E. S., Karamyshev, A. L., Tikhonova, E. B., Wu, I. H., Hudson, H. & Thomas, P. J., Jun 5 2018, In: Cell Reports. 23, 10, p. 2844-2851 8 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Signal Recognition Particle 100%
  • Progranulins 99%
  • RNA Stability 72%
  • Protein Sorting Signals 70%
  • Mutation 55%
  30 Scopus citations
• 2017

  17β-Estradiol dysregulates innate immune responses to Pseudomonas aeruginosa respiratory infection and is modulated by estrogen receptor antagonism

  Abid, S., Xie, S. K., Bose, M., Shaul, P. W., Terada, L. S., Brody, S. L., Thomas, P. J., Katzenellenbogen, J. A., Kim, S. H., Greenberg, D. E. & Jain, R., 2017, In: Infection and immunity. 85, 10, e00422-17.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Pseudomonas Infections 100%
  • estrogen receptors 90%
  • antagonism 83%
  • innate immunity 80%
  • Pseudomonas aeruginosa 78%
  42 Scopus citations

• 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 ^s

  Hudson, R. P., Dawson, J. E., Chong, P. A., Yang, Z., Millen, L., Thomas, P. J., Brouillette, C. G. & Forman-Kay, J. D., Aug 2017, In: Molecular Pharmacology. 92, 2, p. 124-135 12 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • lumacaftor 100%
  • Cystic Fibrosis Transmembrane Conductance Regulator 68%
  • Binding Sites 42%
  • S 10 14%
  • Temperature 13%
  66 Scopus citations
• 2016

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

  Huerta, C., Jiang, X., Trevino, I., Bender, C. F., Ferguson, D. A., Probst, B., Swinger, K. K., Stoll, V. S., Thomas, P. J., Dulubova, I., Visnick, M. & Wigley, W. C., Nov 1 2016, In: Biochimica et Biophysica Acta - General Subjects. 1860, 11, p. 2537-2552 16 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Antioxidant Agent 100%
  • Antioxidants 97%
  • Inflammation 77%
  • Triterpenes 68%
  • Triterpenoid 52%
  43 Scopus citations

• N-alpha-acetyltransferases and regulation of CFTR expression

  Vetter, A. J., Karamyshev, A. L., Patrick, A. E., Hudson, H. & Thomas, P. J., May 2016, In: PloS one. 11, 5, e0155430.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • N-Terminal Acetyltransferases 100%
  • Cystic Fibrosis Transmembrane Conductance Regulator 67%
  • Acetylation 13%
  • Proteins 6%
  • Mutation 4%
  4 Scopus citations
• 2014

  Inefficient SRP interaction with a nascent chain triggers a MRNA quality control pathway

  Karamyshev, A. L., Patrick, A. E., Karamysheva, Z. N., Griesemer, D. S., Hudson, H., Tjon-Kon-Sang, S., Nilsson, I., Otto, H., Liu, Q., Rospert, S., Von Heijne, G., Johnson, A. E. & Thomas, P. J., 2014, In: Cell. 156, 1-2, p. 146-157 12 p.

  Research output: Contribution to journal › Article › peer-review

  • Signal Recognition Particle 100%
  • Quality Control 63%
  • RNA Stability 54%
  • Protein Sorting Signals 35%
  • Messenger RNA 28%
  68 Scopus citations

• The bip molecular chaperone plays multiple roles during the biogenesis of torsina, an aaa atpase associated with the neurological disease early-onset torsion dystonia

  Zacchi, L. F., Wu, H. C., Bell, S. L., Millen, L., Paton, A. W., Paton, J. C., Thomas, P. J., Zolkiewski, M. & Brodsky, J. L., 2014, In: Journal of Biological Chemistry. 289, 18, p. 12727-12747 21 p.

  Research output: Contribution to journal › Article › peer-review

  • Dystonia musculorum deformans type 1 100%
  • Dystonia Musculorum Deformans 95%
  • ATPases Associated with Diverse Cellular Activities 84%
  • Molecular Chaperones 70%
  • Glycosylation 58%
  20 Scopus citations
• 2013

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

  Sosnay, P. R., Siklosi, K. R., Van Goor, F., Kaniecki, K., Yu, H., Sharma, N., Ramalho, A. S., Amaral, M. D., Dorfman, R., Zielenski, J., Masica, D. L., Karchin, R., Millen, L., Thomas, P. J., Patrinos, G. P., Corey, M., Lewis, M. H., Rommens, J. M., Castellani, C., Penland, C. M., & 1 othersCutting, G. R., Oct 2013, In: Nature genetics. 45, 10, p. 1160-1167 8 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Regulator Genes 82%
  • Cystic Fibrosis 77%
  • Penetrance 30%
  • North America 28%
  477 Scopus citations

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

  Bozoky, Z., Krzeminski, M., Muhandiram, R., Birtley, J. R., Al-Zahrani, A., Thomas, P. J., Frizzell, R. A., Ford, R. C. & Forman-Kay, J. D., Nov 19 2013, In: Proceedings of the National Academy of Sciences of the United States of America. 110, 47, p. E4427-E4436

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Chloride Channels 99%
  • Nucleic Acid Regulatory Sequences 91%
  • Phosphorylation 41%
  • Sulfate Transporters 34%
  121 Scopus citations
• 2012

  Development of CFTR structure

  Patrick, A. E. & Thomas, P. J., 2012, In: Frontiers in Pharmacology. 3 SEP, Article 162.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • ATP-Binding Cassette Transporters 23%
  • Mutation 16%
  • Molecular Structure 11%
  • Sequence Deletion 11%
  14 Scopus citations

• Requirements for efficient correction of Δf508 CFTR revealed by analyses of evolved sequences

  Mendoza, J. L., Schmidt, A., Li, Q., Nuvaga, E., Barrett, T., Bridges, R. J., Feranchak, A. P., Brautigam, C. A. & Thomas, P. J., Jan 20 2012, In: Cell. 148, 1-2, p. 164-174 11 p.

  Research output: Contribution to journal › Article › peer-review

  • Conductance 100%
  • Sequence Analysis 97%
  • Mutation 88%
  • Cystic Fibrosis Transmembrane Conductance Regulator 66%
  • Cystic Fibrosis 51%
  220 Scopus citations

• TDP-43 identified from a genome wide RNAi screen for SOD1 regulators

  Somalinga, B. R., Day, C. E., Wei, S., Roth, M. G. & Thomas, P. J., Apr 26 2012, In: PloS one. 7, 4, e35818.

  Research output: Contribution to journal › Article › peer-review

  • Superoxide Dismutase-1 100%
  • amyotrophic lateral sclerosis 84%
  • Superoxide 77%
  • RNA Interference 75%
  • superoxide dismutase 62%
  12 Scopus citations
• 2011

  Alteration of CFTR transmembrane span integration by disease-causing mutations

  Patrick, A. E., Karamyshev, A. L., Millen, L. & Thomas, P. J., Dec 1 2011, In: Molecular biology of the cell. 22, 23, p. 4461-4471 11 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Endoplasmic Reticulum 76%
  • Mutation 44%
  • Proteins 29%
  • Glycine 26%
  23 Scopus citations

• A screen to identify cellular modulators of soluble levels of an amyotrophic lateral sclerosis (ALS)-causing mutant SOD1

  Somalinga, B. R., Miller, G. A., Malik, H. T., Wigley, W. C. & Thomas, P. J., Oct 2011, In: Journal of Biomolecular Screening. 16, 9, p. 974-985 12 p.

  Research output: Contribution to journal › Article › peer-review

  • Amyotrophic Lateral Sclerosis 100%
  • Solubility 93%
  • Complementary DNA 41%
  • Protein 21%
  • Clone Cells 21%
  3 Scopus citations

• Biochemical and biophysical approaches to probe CFTR structure.

  Schmidt, A., Mendoza, J. L. & Thomas, P. J., 2011, In: Methods in molecular biology (Clifton, N.J.). 741, p. 365-376 12 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis 100%
  • Mutation 24%
  • Genetic Suppression 20%
  • Fluids and Secretions 19%
  • ATP-Binding Cassette Transporters 17%
  4 Scopus citations

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

  Peters, K. W., Okiyoneda, T., Balch, W. E., Braakman, I., Brodsky, J. L., Guggino, W. B., Penland, C. M., Pollard, H. B., Sorscher, E. J., Skach, W. R., Thomas, P. J., Lukacs, G. L. & Frizzell, R. A., 2011, In: Methods in molecular biology (Clifton, N.J.). 742, p. 335-353 19 p.

  Research output: Contribution to journal › Article › peer-review

  • Antibodies 100%
  • Drug Discovery 92%
  • Cystic Fibrosis 88%
  • Drug Development 85%
  • Proteins 66%
  27 Scopus citations

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

  Yang, D., Li, Q., So, I., Huang, C. L., Ando, H., Mizutani, A., Seki, G., Mikoshiba, K., Thomas, P. J. & Muallem, S., Mar 1 2011, In: Journal of Clinical Investigation. 121, 3, p. 956-965 10 p.

  Research output: Contribution to journal › Article › peer-review

  • PAS domain kinases 100%
  • Phosphotransferases 41%
  • Protein Phosphatase 1 29%
  • Fluids and Secretions 29%
  • Sodium-Bicarbonate Symporters 18%
  83 Scopus citations
• 2010

  Abnormal neurites containing C-terminally truncated α-synuclein are present in Alzheimer's disease without conventional lewy body pathology

  Lewis, K. A., Su, Y., Jou, O., Ritchie, C., Foong, C., Hynan, L. S., White, C. L., Thomas, P. J. & Hatanpaa, K. J., Dec 2010, In: American Journal of Pathology. 177, 6, p. 3037-3050 14 p.

  Research output: Contribution to journal › Article › peer-review

  • Synucleins 100%
  • Lewy Bodies 85%
  • Neurites 73%
  • Lewy Body Disease 67%
  • Alzheimer Disease 51%
  32 Scopus citations

• Accelerated formation of α-synuclein oligomers by concerted action of the 20s proteasome and familial parkinson mutations

  Lewis, K. A., Yaeger, A., Demartino, G. N. & Thomas, P. J., Feb 2010, In: Journal of Bioenergetics and Biomembranes. 42, 1, p. 85-95 11 p.

  Research output: Contribution to journal › Article › peer-review

  • Synucleins 100%
  • Proteasome Endopeptidase Complex 68%
  • Mutation 36%
  • Parkinson Disease 30%
  • Amyloid 28%
  19 Scopus citations

• A unique redox-sensing sensor II motif in torsina plays a critical role in nucleotide and partner binding

  Zhu, L., Millen, L., Mendoza, J. L. & Thomas, P. J., Nov 26 2010, In: Journal of Biological Chemistry. 285, 48, p. 37271-37280 10 p.

  Research output: Contribution to journal › Article › peer-review

  • Oxidation-Reduction 100%
  • Polypeptide 86%
  • Nucleotide 85%
  • Nucleotides 84%
  • Dystonia 73%
  44 Scopus citations

• NMR evidence for differential phosphorylation-dependent interactions in WT and DF508 CFTR

  Kanelis, V., Hudson, R. P., Thibodeau, P. H., Thomas, P. J. & Forman-Kay, J. D., Jan 6 2010, In: EMBO Journal. 29, 1, p. 263-277 15 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Phosphorylation 54%
  • Nucleotides 38%
  • Nucleic Acid Regulatory Sequences 36%
  • Chloride Channels 19%
  85 Scopus citations

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

  Hutt, D. M., Herman, D., Rodrigues, A. P. C., Noel, S., Pilewski, J. M., Matteson, J., Hoch, B., Kellner, W., Kelly, J. W., Schmidt, A., Thomas, P. J., Matsumura, Y., Skach, W. R., Gentzsch, M., Riordan, J. R., Sorscher, E. J., Okiyoneda, T., Yates, J. R., Lukacs, G. L., Frizzell, R. A., & 3 othersManning, G., Gottesfeld, J. M. & Balch, W. E., Jan 2010, In: Nature chemical biology. 6, 1, p. 25-33 9 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Histone Deacetylase Inhibitors 93%
  • Histone Deacetylases 92%
  • Cystic Fibrosis 77%
  • Vorinostat 56%
  223 Scopus citations

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

  Kalid, O., Mense, M., Fischman, S., Shitrit, A., Bihler, H., Ben-Zeev, E., Schutz, N., Pedemonte, N., Thomas, P. J., Bridges, R. J., Wetmore, D. R., Marantz, Y. & Senderowitz, H., Dec 2010, In: Journal of Computer-Aided Molecular Design. 24, 12, p. 971-991 21 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Molecules 100%
  • Computer Simulation 97%
  • Binding sites 95%
  • Screening 95%
  • screening 88%
  83 Scopus citations

• The cystic fibrosis-causing mutation ΔF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis

  Thibodeau, P. H., Richardson, J. M., Wang, W., Millen, L., Watson, J., Mendoza, J. L., Du, K., Fischman, S., Senderowitz, H., Lukacs, G. L., Kirk, K. & Thomas, P. J., Nov 12 2010, In: Journal of Biological Chemistry. 285, 46, p. 35825-35835 11 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Genetic Suppression 83%
  • Cystic Fibrosis 77%
  • Mutation 67%
  • Nucleotides 32%
  146 Scopus citations

• The primary folding defect and rescue of δf508 CFTR emerge during translation of the mutant domain

  Hoelen, H., Kleizen, S., Schmidt, A., Richardson, J., Charitou, P., Thomas, P. J. & Braakman, I., 2010, In: PloS one. 5, 11, e15458.

  Research output: Contribution to journal › Article › peer-review

  • cystic fibrosis 100%
  • Drug Target 83%
  • Cystic Fibrosis 71%
  • translation (genetics) 63%
  • Mutation 61%
  71 Scopus citations
• 2008

  Congenital chloride-losing diarrhea causing mutations in the STAS domain result in misfolding and mistrafficking of SLC26A3

  Dorwart, M. R., Shcheynikov, N., Baker, J. M. R., Forman-Kay, J. D., Muallem, S. & Thomas, P. J., Mar 28 2008, In: Journal of Biological Chemistry. 283, 13, p. 8711-8722 12 p.

  Research output: Contribution to journal › Article › peer-review

  • Congenital chloride diarrhea 100%
  • Sulfate Transporters 95%
  • Sigma Factor 73%
  • Mutation 46%
  • Solute 45%
  59 Scopus citations

• Solubilizing Mutations Used to Crystallize One CFTR Domain Attenuate the Trafficking and Channel Defects Caused by the Major Cystic Fibrosis Mutation

  Pissarra, L. S., Farinha, C. M., Xu, Z., Schmidt, A., Thibodeau, P. H., Cai, Z., Thomas, PJ., Sheppard, D. N. & Amaral, M. D., Jan 25 2008, In: Chemistry and Biology. 15, 1, p. 62-69 8 p.

  Research output: Contribution to journal › Article › peer-review

  • Solubilizing 100%
  • Cystic Fibrosis 83%
  • Conductance 80%
  • Mutation 71%
  • Crystallization 20%
  67 Scopus citations

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

  Zhu, L., Wrabl, J. O., Hayashi, A. P., Rose, L. S. & Thomas, P. J., Aug 2008, In: Molecular biology of the cell. 19, 8, p. 3599-3612 14 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • AAA Proteins 100%
  • Disulfides 64%
  • Oxidation-Reduction 58%
  • Cysteine 57%
  • Nucleotides 48%
  48 Scopus citations
• 2007

  CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices

  Baker, J. M. R., Hudson, R. P., Kanelis, V., Choy, W. Y., Thibodeau, P. H., Thomas, P. J. & Forman-Kay, J. D., Aug 2007, In: Nature Structural and Molecular Biology. 14, 8, p. 738-745 8 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Nucleic Acid Regulatory Sequences 91%
  • Phosphorylation 41%
  • Nucleotides 32%
  • Hydrolysis 18%
  238 Scopus citations

• Hook2 contributes to aggresome formation

  Szebenyi, G., Wigley, W. C., Hall, B., Didier, A., Yu, M., Thomas, P. & Krämer, H., May 31 2007, In: BMC Cell Biology. 8, 19.

  Research output: Contribution to journal › Article › peer-review

  • Microtubules 100%
  • Organelles 73%
  • Dyneins 67%
  • Centrosome 64%
  • Proteins 37%
  40 Scopus citations
• 2006

  ATP Binding and ATP Hydrolysis Play Distinct Roles in the Function of 26S Proteasome

  Liu, C. W., Li, X., Thompson, D., Wooding, K., Chang, T. L., Tang, Z., Yu, H., Thomas, P. J. & DeMartino, G. N., Oct 6 2006, In: Molecular cell. 24, 1, p. 39-50 12 p.

  Research output: Contribution to journal › Article › peer-review

  • ATP dependent 26S protease 100%
  • Hydrolysis 66%
  • Adenosine Triphosphate 55%
  • Proteasome Endopeptidase Complex 31%
  • Proteolysis 28%
  149 Scopus citations

• Coupling modes and stoichiometry of Cl^-/HCO₃^- exchange by slc26a3 and slc26a6

  Shcheynikov, N., Wang, Y., Park, M., Ko, S. B. H., Dorwart, M., Naruse, S., Thomas, P. J. & Muallem, S., May 2006, In: Journal of General Physiology. 127, 5, p. 511-524 14 p.

  Research output: Contribution to journal › Article › peer-review

  • Membrane Potentials 100%
  • Oocytes 47%
  • Xenopus laevis 29%
  • Protein Isoforms 20%
  • Kidney 13%
  159 Scopus citations

• Slc26a6 regulates CFTR activity in vivo to determine pancreatic duct HCO₃^- secretion: Relevance to cystic fibrosis

  Wang, Y., Soyombo, A. A., Shcheynikov, N., Zeng, W., Dorwart, M., Marino, C. R., Thomas, P. J. & Muallem, S., Nov 1 2006, In: EMBO Journal. 25, 21, p. 5049-5057 9 p.

  Research output: Contribution to journal › Article › peer-review

  • Fluids and Secretions 100%
  • Silencing RNA 95%
  • Pancreatic Ducts 84%
  • Cystic Fibrosis 72%
  • 3-((3-trifluoromethyl)phenyl)-5-((3-carboxyphenyl)methylene)-2-thioxo-4-thiazolidinone 38%
  129 Scopus citations
• 2005

  Aggresome formation.

  Corboy, M. J., Thomas, P. J. & Wigley, W. C., 2005, In: Methods in molecular biology (Clifton, N.J.). 301, p. 305-327 23 p.

  Research output: Contribution to journal › Article › peer-review

  • Proteasome Endopeptidase Complex 100%
  • Ubiquitin 64%
  • Quality Control 46%
  • Centrosome 39%
  • Proteins 35%
  48 Scopus citations

• A precipitating role for truncated α-synuclein and the proteasome in α-synuclein aggregation: Implications for pathogenesis of parkinson disease

  Liu, C. W., Giasson, B. I., Lewis, K. A., Lee, V. M., DeMartino, G. N. & Thomas, P. J., Jun 17 2005, In: Journal of Biological Chemistry. 280, 24, p. 22670-22678 9 p.

  Research output: Contribution to journal › Article › peer-review

  • Synucleins 100%
  • Proteasome Endopeptidase Complex 68%
  • Parkinson Disease 55%
  • Protein 27%
  • Oxidative Stress 16%
  228 Scopus citations

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

  Liu, C. W., Strickland, E., Demartino, G. N. & Thomas, P. J., 2005, In: Methods in molecular biology (Clifton, N.J.). 301, p. 71-81 11 p.

  Research output: Contribution to journal › Article › peer-review

  • PA700 proteasome activator 100%
  • ATP dependent 26S protease 67%
  • Polyubiquitin 35%
  • Proteasome Endopeptidase Complex 25%
  • Adenosine Triphosphatases 24%
  12 Scopus citations

• Side chain and backbone contributions of Phe508 to CFTR folding

  Thibodeau, P. H., Brautigam, C. A., Machius, M. & Thomas, P. J., Jan 2005, In: Nature Structural and Molecular Biology. 12, 1, p. 10-16 7 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Missense Mutation 41%
  • Cystic Fibrosis 38%
  • Membrane Proteins 16%
  • Nucleotides 16%
  153 Scopus citations
• 2004

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

  Lee, S. F., Shah, S., Yu, C., Wigley, W. C., Li, H., Lim, M., Pedersen, K., Han, W., Thomas, P., Lundkvist, J., Hao, Y. H. & Yu, G., Feb 6 2004, In: Journal of Biological Chemistry. 279, 6, p. 4144-4152 9 p.

  Research output: Contribution to journal › Article › peer-review

  • Presenilins 100%
  • Amyloid Precursor Protein Secretases 86%
  • Mutation 58%
  • Protein Hydrolysis 50%
  • Caenorhabditis elegans 38%
  100 Scopus citations

• Cystic fibrosis: Recent structural insights

  Dorwart, M., Thibodeau, P. & Thomas, P., Aug 2004, In: Journal of Cystic Fibrosis. 3, SUPPL. 2, p. 91-94 4 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Cystic Fibrosis 77%
  • Nucleotides 42%
  • ATP-Binding Cassette Transporters 31%
  • Mutation 29%
  14 Scopus citations

• Dynamic control of cystic fibrosis transmembrane conductance regulator Cl^-/HCO₃^- selectivity by external Cl ^-

  Shcheynikov, N., Kim, K. H., Kim, K. M., Dorwart, M. R., Ko, S. B. H., Goto, H., Naruse, S., Thomas, P. J. & Muallem, S., May 21 2004, In: Journal of Biological Chemistry. 279, 21, p. 21857-21865 9 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Membrane Potential 86%
  • hydroxide ion 75%
  • Membrane Potentials 64%
  • Permeability 25%
  89 Scopus citations

• Gating of CFTR by the STAS domain of SLC26 transporters

  Ko, S. B. H., Zeng, W., Dorwart, M. R., Luo, X., Kim, K. H., Millen, L., Goto, H., Naruse, S., Soyombo, A., Thomas, P. J. & Muallem, S., Apr 2004, In: Nature cell biology. 6, 4, p. 343-350 8 p.

  Research output: Contribution to journal › Article › peer-review

  • Bicarbonates 100%
  • Chlorides 97%
  • Cystic Fibrosis 59%
  • Chloride-Bicarbonate Antiporters 46%
  • Membranes 36%
  377 Scopus citations

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

  Lewis, H. A., Buchanan, S. G., Burley, S. K., Conners, K., Dickey, M., Dorwart, M., Fowler, R., Gao, X., Guggino, W. B., Hendrickson, W. A., Hunt, J. F., Kearins, M. C., Lorimer, D., Maloney, P. C., Post, K. W., Rajashankar, K. R., Rutter, M. E., Sauder, J. M., Shriver, S., Thibodeau, P. H., & 4 othersThomas, P. J., Zhang, M., Zhao, X. & Emtage, S., Jan 28 2004, In: EMBO Journal. 23, 2, p. 282-293 12 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Nucleotides 64%
  • Nucleotide 43%
  • Adenosine Triphosphate 27%
  • Mutation 22%
  343 Scopus citations

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

  Lee, R. J., Liu, C. W., Harty, C., McCracken, A. A., Latterich, M., Römisch, K., DeMartino, G. N., Thomas, P. J. & Brodsky, J. L., Jun 2 2004, In: EMBO Journal. 23, 11, p. 2206-2215 10 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Endoplasmic Reticulum-Associated Degradation 100%
  • Proteasome Endopeptidase Complex 70%
  • Polypeptide 55%
  • Cytosol 43%
  • Endoplasmic Reticulum 43%
  101 Scopus citations
• 2003

  Assessment of protein folding/solubility in live cells.

  Stidham, R. D., Wigley, W. C., Hunt, J. F. & Thomas, P. J., 2003, In: Methods in molecular biology (Clifton, N.J.). 205, p. 155-169 15 p.

  Research output: Contribution to journal › Article › peer-review
  4 Scopus citations

• Endoproteolytic activity of the proteasome

  Liu, C. W., Corboy, M. J., DeMartino, G. N. & Thomas, P. J., Jan 17 2003, In: Science. 299, 5605, p. 408-411 4 p.

  Research output: Contribution to journal › Article › peer-review

  • Proteasome Endopeptidase Complex 100%
  • Catalytic Domain 28%
  • Transcription Factors 24%
  • Proteins 23%
  • Peptides 20%
  359 Scopus citations

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

  Zhang, X. M., Wang, X. T., Yue, H., Leung, S. W., Thibodeau, P. H., Thomas, P. J. & Guggino, S. E., Dec 19 2003, In: Journal of Biological Chemistry. 278, 51, p. 51232-51242 11 p.

  Research output: Contribution to journal › Article › peer-review

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Solute 64%
  • Inositol 29%
  • Cell Membrane 19%
  • trimethyloxamine 13%
  76 Scopus citations
• 2002

  A molecular mechanism for aberrant CFTR-dependent HCO₃^- transport in cystic fibrosis

  Ko, S. B. H., Shcheynikov, N., Choi, J. Y., Luo, X., Ishibashi, K., Thomas, P. J., Kim, J. Y., Kim, K. H., Lee, M. G., Naruse, S. & Muallem, S., Nov 1 2002, In: EMBO Journal. 21, 21, p. 5662-5672 11 p.

  Research output: Contribution to journal › Article › peer-review

  Open Access

  • Cystic Fibrosis Transmembrane Conductance Regulator 100%
  • Cystic Fibrosis 77%
  • Conductance 75%
  • Reaction Stoichiometry 18%
  • Congenital chloride diarrhea 18%
  289 Scopus citations