Physiology - Division T

Research Output

2019

CFTR modulator theratyping: Current status, gaps and future directions

Clancy, J. P., Cotton, C. U., Donaldson, S. H., Solomon, G. M., VanDevanter, D. R., Boyle, M. P., Gentzsch, M., Nick, J. A., Illek, B., Wallenburg, J. C., Sorscher, E. J., Amaral, M. D., Beekman, J. M., Naren, A. P., Bridges, R. J., Thomas, P. J., Cutting, G., Rowe, S., Durmowicz, A. G., Mense, M. & 8 others, Boeck, K. D., Skach, W., Penland, C., Joseloff, E., Bihler, H., Mahoney, J., Borowitz, D. & Tuggle, K. L., Jan 2019, In : Journal of Cystic Fibrosis. 18, 1, p. 22-34 13 p.

Research output: Contribution to journalReview article

38 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 journalArticle

20 Scopus citations

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., Jan 1 2018, (Accepted/In press) In : Journal of Cystic Fibrosis.

Research output: Contribution to journalArticle

11 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 journalArticle

9 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., Jan 1 2018, (Accepted/In press) In : Cell Reports.

Research output: Contribution to journalArticle

8 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., Jan 1 2017, In : Infection and immunity. 85, 10, e00422-17.

Research output: Contribution to journalArticle

15 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 journalArticle

26 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 journalArticle

20 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 1 2016, In : PLoS One. 11, 5, e0155430.

Research output: Contribution to journalArticle

3 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., Jan 27 2014, In : Cell. 156, 1-2, p. 146-157 12 p.

Research output: Contribution to journalArticle

34 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., Jan 1 2014, In : Journal of Biological Chemistry. 289, 18, p. 12727-12747 21 p.

Research output: Contribution to journalArticle

14 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 others, Cutting, G. R., Oct 1 2013, In : Nature genetics. 45, 10, p. 1160-1167 8 p.

Research output: Contribution to journalArticle

326 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 journalArticle

82 Scopus citations
2012

Development of CFTR structure

Patrick, A. E. & Thomas, P. J., Oct 10 2012, In : Frontiers in Pharmacology. 3 SEP, Article 162.

Research output: Contribution to journalArticle

12 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 journalArticle

165 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 journalArticle

10 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 journalArticle

12 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 1 2011, In : Journal of Biomolecular Screening. 16, 9, p. 974-985 12 p.

Research output: Contribution to journalArticle

3 Scopus citations

Biochemical and biophysical approaches to probe CFTR structure.

Schmidt, A., Mendoza, J. L. & Thomas, P. J., Sep 7 2011, In : Methods in molecular biology (Clifton, N.J.). 741, p. 365-376 12 p.

Research output: Contribution to journalArticle

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., Sep 13 2011, In : Methods in molecular biology (Clifton, N.J.). 742, p. 335-353 19 p.

Research output: Contribution to journalArticle

21 Scopus citations

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

Mendoza, J. L., Schmidt, A. & Thomas, P. J., Sep 7 2011, In : Methods in molecular biology (Clifton, N.J.). 741, p. 321-327 7 p.

Research output: Contribution to journalEditorial

2 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 journalArticle

65 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 journalArticle

20 Scopus citations
16 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 journalArticle

36 Scopus citations

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

Balch, W. E., Braakman, I., Brodsky, J., Frizzell, R., Guggino, W., Lukacs, G. L., Penland, C., Pollard, H., Skach, W., Sorscher, E. & Thomas, P., Jul 2 2010, Protein Misfolding Diseases: Current and Emerging Principles and Therapies. John Wiley and Sons, p. 425-452 28 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

1 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 journalArticle

68 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 others, Manning, G., Gottesfeld, J. M. & Balch, W. E., Jan 2010, In : Nature chemical biology. 6, 1, p. 25-33 9 p.

Research output: Contribution to journalArticle

183 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 1 2010, In : Journal of Computer-Aided Molecular Design. 24, 12, p. 971-991 21 p.

Research output: Contribution to journalArticle

70 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 journalArticle

110 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., Dec 10 2010, In : PloS one. 5, 11, e15458.

Research output: Contribution to journalArticle

55 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 journalArticle

54 Scopus citations

Regulatory Interaction between CFTR and the SLC26 Transporters

Shcheynikov, N., Ko, S. B. H., Zeng, W., Choi, J. Y., Dorwart, M. R., Thomas, P. J. & Muallem, S., Oct 7 2008, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. Wiley Blackwell, p. 177-186 10 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

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 journalArticle

61 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 1 2008, In : Molecular biology of the cell. 19, 8, p. 3599-3612 14 p.

Research output: Contribution to journalArticle

43 Scopus citations
2007

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

Mendoza, J. L. & Thomas, P. J., Dec 1 2007, In : Journal of Bioenergetics and Biomembranes. 39, 5-6, p. 499-505 7 p.

Research output: Contribution to journalReview article

37 Scopus citations

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 1 2007, In : Nature Structural and Molecular Biology. 14, 8, p. 738-745 8 p.

Research output: Contribution to journalArticle

204 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 journalArticle

33 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 journalArticle

129 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 1 2006, In : Journal of General Physiology. 127, 5, p. 511-524 14 p.

Research output: Contribution to journalArticle

133 Scopus citations

Discussion

Muallem, S., Oliver, D., Ashmore, J., Alper, S. L., Turner, R. J., Welsh, M. J., Romero, M. F., Kere, J., Thomas, P. J., Quinton, P. M. & Knipper, M., Jan 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. Novartis Foundation, p. 253-260 8 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Discussion

Muallem, S., Case, M., Höglund, P., Quinton, P. M., Kere, J., Lee, M. G., Thomas, P. J., Mount, D. B., Seidler, U., Alper, S. L., Soleimani, M., Chan, H. C. & Welsh, M. J., Dec 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. p. 86-90 5 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Discussion

Welsh, M. J., Rossi, A., Lee, M. G., Markovich, D., Quinton, P. M., Thomas, P. J., Soleimani, M., Alper, S. L., Knipper, M., Romero, M. F., Aronson, P. S., Stewart, A. K., Muallem, S. & Mount, D. B., Jan 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. Novartis Foundation, p. 206-212 7 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Discussion

Muallem, S., Ashmore, J., Quinton, P. M., Alper, S. L., Gray, M. A., Turner, R. J., Thomas, P. J., Romero, M. F., Welsh, M. J., Mount, D. B., Knipper, M., Lee, M. G., Soleimani, M., Aronson, P. S. & Case, M., Jan 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. Novartis Foundation, p. 68-73 6 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Discussion

Welsh, M. J., Knipper, M., Muallem, S., Alper, S. L., Lee, M. G., Everett, L. A., Kere, J., Turner, R. J., Ashmore, J., Mount, D. B., Case, M., Oliver, D., Soleimani, M., Markovich, D., Thomas, P. J., Aronson, P. S., Quinton, P. M. & Chan, H. C., Jan 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. Novartis Foundation, p. 30-41 12 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Discussion

Welsh, M. J., Muallem, S., Lee, M. G., Thomas, P. J., Alper, S. L., Gray, M. A., Soleimani, M., Argent, B. E., Ashmore, J., Case, M., Romero, M. F., Quinton, P. M. & Seidler, U., Dec 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. p. 186-192 7 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Regulatory interaction between CFTR and the SLC26 transporters

Shcheynikov, N., Ko, S. B. H., Zeng, W., Joo, Y. C., Dorwart, M. R., Thomas, P. J. & Muallem, S., Dec 1 2006, Epithelial Anion Transport in Health and Disease: The Role of the SLC26 Transporters Family. p. 177-186 10 p. (Novartis Foundation Symposium; vol. 273).

Research output: Chapter in Book/Report/Conference proceedingConference contribution

47 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 journalArticle

100 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 journalArticle

43 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 journalArticle

181 Scopus citations