PubRank

Philip J Thomas

Author PubWeight™ 73.57‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 ATP binding to the motor domain from an ABC transporter drives formation of a nucleotide sandwich dimer. Mol Cell 2002 4.79
2 Gating of CFTR by the STAS domain of SLC26 transporters. Nat Cell Biol 2004 3.43
3 Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J 2003 3.35
4 Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet 2013 2.91
5 Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis. Nat Chem Biol 2009 2.70
6 Cooperative, ATP-dependent association of the nucleotide binding cassettes during the catalytic cycle of ATP-binding cassette transporters. J Biol Chem 2002 2.64
7 Endoproteolytic activity of the proteasome. Science 2002 2.56
8 Side chain and backbone contributions of Phe508 to CFTR folding. Nat Struct Mol Biol 2004 2.43
9 ATP binding and ATP hydrolysis play distinct roles in the function of 26S proteasome. Mol Cell 2006 2.26
10 A molecular mechanism for aberrant CFTR-dependent HCO(3)(-) transport in cystic fibrosis. EMBO J 2002 2.11
11 CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices. Nat Struct Mol Biol 2007 1.98
12 Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. Chem Biol 2008 1.97
13 A protein sequence that can encode native structure by disfavoring alternate conformations. Nat Struct Biol 2002 1.85
14 Requirements for efficient correction of ΔF508 CFTR revealed by analyses of evolved sequences. Cell 2012 1.81
15 Coupling modes and stoichiometry of Cl-/HCO3- exchange by slc26a3 and slc26a6. J Gen Physiol 2006 1.73
16 A precipitating role for truncated alpha-synuclein and the proteasome in alpha-synuclein aggregation: implications for pathogenesis of Parkinson disease. J Biol Chem 2005 1.65
17 Slc26a6 regulates CFTR activity in vivo to determine pancreatic duct HCO3- secretion: relevance to cystic fibrosis. EMBO J 2006 1.56
18 The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis. J Biol Chem 2010 1.55
19 Congenital chloride-losing diarrhea causing mutations in the STAS domain result in misfolding and mistrafficking of SLC26A3. J Biol Chem 2008 1.43
20 Uncoupling retro-translocation and degradation in the ER-associated degradation of a soluble protein. EMBO J 2004 1.38
21 Dynamic control of cystic fibrosis transmembrane conductance regulator Cl(-)/HCO3(-) selectivity by external Cl(-). J Biol Chem 2004 1.28
22 The torsin-family AAA+ protein OOC-5 contains a critical disulfide adjacent to Sensor-II that couples redox state to nucleotide binding. Mol Biol Cell 2008 1.25
23 Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator. J Biol Chem 2003 1.23
24 IRBIT governs epithelial secretion in mice by antagonizing the WNK/SPAK kinase pathway. J Clin Invest 2011 1.18
25 Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening. J Comput Aided Mol Des 2010 1.17
26 The primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domain. PLoS One 2010 1.17
27 Conformational remodeling of proteasomal substrates by PA700, the 19 S regulatory complex of the 26 S proteasome. J Biol Chem 2002 1.15
28 Building an understanding of cystic fibrosis on the foundation of ABC transporter structures. J Bioenerg Biomembr 2007 1.14
29 NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR. EMBO J 2009 1.12
30 Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions. Proc Natl Acad Sci U S A 2013 1.06
31 The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3. J Biol Chem 2002 1.06
32 Aggresome formation. Methods Mol Biol 2005 1.06
33 Regulatory interaction between CFTR and the SLC26 transporters. Novartis Found Symp 2006 1.04
34 A unique redox-sensing sensor II motif in TorsinA plays a critical role in nucleotide and partner binding. J Biol Chem 2010 1.04
35 Nucleotide binding domain interactions during the mechanochemical reaction cycle of ATP-binding cassette transporters. J Bioenerg Biomembr 2005 0.93
36 Mutations that change the position of the putative gamma-phosphate linker in the nucleotide binding domains of CFTR alter channel gating. J Biol Chem 2002 0.91
37 Recognition and processing of misfolded proteins by PA700, the 19S regulatory complex of the 26S proteasome. Methods Mol Biol 2005 0.87
38 Accelerated formation of alpha-synuclein oligomers by concerted action of the 20S proteasome and familial Parkinson mutations. J Bioenerg Biomembr 2010 0.84
39 Abnormal neurites containing C-terminally truncated alpha-synuclein are present in Alzheimer's disease without conventional Lewy body pathology. Am J Pathol 2010 0.83
40 Alteration of CFTR transmembrane span integration by disease-causing mutations. Mol Biol Cell 2011 0.82
41 Assessment of protein folding/solubility in live cells. Methods Mol Biol 2003 0.80
42 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 Biol Chem 2014 0.79
43 CFTR Folding Consortium: methods available for studies of CFTR folding and correction. Methods Mol Biol 2011 0.79
44 Biochemical and biophysical approaches to probe CFTR structure. Methods Mol Biol 2011 0.78
45 TDP-43 identified from a genome wide RNAi screen for SOD1 regulators. PLoS One 2012 0.78
46 Development of CFTR Structure. Front Pharmacol 2012 0.77
47 Doxazosin and serotonin (5-HT) receptor (1A, 2A, and 4) antagonists inhibit 5-HT-mediated human cavernosal contraction. J Androl 2006 0.77
48 Introduction to section IV: biophysical methods to approach CFTR structure. Methods Mol Biol 2011 0.76
49 A screen to identify cellular modulators of soluble levels of an amyotrophic lateral sclerosis (ALS)-causing mutant SOD1. J Biomol Screen 2011 0.76
50 CFTR degradation and aggregation. Methods Mol Med 2002 0.76
51 In vitro CFTR folding assays. Methods Mol Med 2002 0.75