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Bear Lab

Publications

2011

Wellhauser L, Luna-Chavez C, D'Antonio C, Tainer J, Bear CE. ATP induces conformational changes in the carboxyl-terminal region of ClC-5. J Biol Chem. 2011 Feb 25;286(8):6733-41.PMID:21173145
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2010

Lamhonwah AM, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I. Ann Neurol.  Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise. 2010 Jun;67(6):802-8. PMID: 20517942
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Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE. A chemical corrector modifies the channel function of F508del-CFTR. Mol Pharmacol. 2010 Sep 1;78(3):411-8. PMID: 20501743
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Cortez MA, Li C, Whitehead SN, Dhani SU, D'Antonio C, Huan LJ, Bennett SA, Snead OC 3rd, Bear CE. Disruption of ClC-2 expression is associated with progressive neurodegeneration in aging mice. Neuroscience. 2010 Apr 28;167(1):154-62. PMID: 20116415
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Wellhauser L, D'Antonio C, Bear CE. ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5. Pflugers Arch. 2010 Jul;460(2):543-57. Review. PMID: 20049483
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2009

Cheung JC, Kim Chiaw P, Deber CM, Bear CE. A novel method for monitoring the cytosolic delivery of peptide cargo. J Control Release. 2009 Jul 1;137(1):2-7. Epub 2009 Mar 12. PubMed PMID: 19285529.
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Wellhauser L, Chiaw PK, Pasyk S, Li C, Ramjeesingh M, Bear CE. A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability. Mol Pharmacol. 2009 Jun;75(6):1430-8. Epub 2009 Apr 1. PubMed PMID: 19339490.
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Chiaw PK, Huan LJ, Gagnon S, Ly D, Sweezey N, Rotin D, Deber CM, Bear CE. Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs. Chem Biol. 2009 May 29;16(5):520-30. PubMed PMID: 19477416.
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Mohammad-Panah R, Wellhauser L, Steinberg BE, Wang Y, Huan LJ, Liu XD, Bear CE. An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice. J Cell Sci. 2009 Apr 15;122(Pt 8):1229-37. PubMed PMID: 19339555.
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Pasyk S, Li C, Ramjeesingh M, Bear CE. Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease. Biochem J. 2009 Feb 15;418(1):185-90. PubMed PMID: 18945216.
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2008

Cheung JC, Kim Chiaw P, Pasyk S, Bear CE. Molecular basis for the ATPase activity of CFTR. Arch Biochem Biophys. 2008 Aug 1;476(1):95-100. Epub 2008 Apr 8. Review. PubMed PMID: 18417076.
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Ramjeesingh M, Ugwu F, Stratford FL, Huan LJ, Li C, Bear CE. The intact CFTR protein mediates ATPase rather than adenylate kinase activity. Biochem J. 2008 Jun 1;412(2):315-21. PubMed PMID: 18241200.
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Mumbengegwi DR, Li Q, Li C, Bear CE, Engelhardt JF. Evidence for a superoxide permeability pathway in endosomal membranes. Mol Cell Biol. 2008 Jun;28(11):3700-12. Epub 2008 Mar 31. PubMed PMID: 18378695; PubMed Central PMCID: PMC2423302.
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Payandeh J, Li C, Ramjeesingh M, Poduch E, Bear CE, Pai EF. Probing structure-function relationships and gating mechanisms in the CorA Mg2+ transport system. J Biol Chem. 2008 Apr 25;283(17):11721-33. Epub 2008 Feb 14. PubMed PMID: 18276588.
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Dhani SU, Kim Chiaw P, Huan LJ, Bear CE. ATP depletion inhibits the endocytosis of ClC-2. J Cell Physiol. 2008 Jan;214(1):273-80. PubMed PMID: 17620322.
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2007

Stratford FL, Ramjeesingh M, Cheung JC, Huan LJ, Bear CE. The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer. Biochem J. 2007 Jan 15;401(2):581-6. PubMed PMID: 16989640; PubMed Central PMCID: PMC1820796.
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2006

Wellhauser L, Kuo HH, Stratford FL, Ramjeesingh M, Huan LJ, Luong W, Li C, Deber CM, Bear CE. Nucleotides bind to the C-terminus of ClC-5. Biochem J. 2006 Sep 1;398(2):289-94. PubMed PMID: 16686597; PubMed Central PMCID: PMC1550301.
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Ramjeesingh M, Li C, She YM, Bear CE. Evaluation of the membrane-spanning domain of ClC-2. Biochem J. 2006 Jun 15;396(3):449-60. PubMed PMID: 16526942; PubMed Central PMCID: PMC1482819.
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2005

Dhani SU, Bear CE. Role of intramolecular and intermolecular interactions in ClC channel and transporter function. Pflugers Arch. 2006 Mar;451(6):708-15. Epub 2005 Sep 16. PubMed PMID: 16167151.
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2004

Kidd JF, Ramjeesingh M, Stratford F, Huan LJ, Bear CE. A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity. J Biol Chem. 2004 Oct 1;279(40):41664-9. Epub 2004 Jul 28. PubMed PMID: 15284228.
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Kogan I, Ramjeesingh M, Bear CE. ATPase assay of purified, reconstituted CFTR protein. J Cyst Fibros. 2004 Aug;3 Suppl 2:133-4. PubMed PMID: 15463945.
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Norez C, Heda GD, Jensen T, Kogan I, Hughes LK, Auzanneau C, Dérand R, Bulteau-Pignoux L, Li C, Ramjeesingh M, Li H, Sheppard DN, Bear CE, Riordan JR, Becq F. Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. J Cyst Fibros. 2004 Aug;3 Suppl 2:119-21. Review. PubMed PMID: 15463942.
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Sheppard DN, Gray MA, Gong X, Sohma Y, Kogan I, Benos DJ, Scott-Ward TS, Chen JH, Li H, Cai Z, Gupta J, Li C, Ramjeesingh M, Berdiev BK, Ismailov II, Bear CE, Hwang TC, Linsdell P, Hug MJ. The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel. J Cyst Fibros. 2004 Aug;3 Suppl 2:101-8. Review. PubMed PMID: 15463939.
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Benos DJ, Berdiev BK, Ismailov II, Ostedgaard LS, Kogan I, Li C, Ramjeesingh M, Bear CE. Methods to study CFTR protein in vitro. J Cyst Fibros. 2004 Aug;3 Suppl 2:79-83. Review. PubMed PMID: 15463933.
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Grimard V, Li C, Ramjeesingh M, Bear CE, Goormaghtigh E, Ruysschaert JM. Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluorescence spectroscopy. J Biol Chem. 2004 Feb 3;279(7):5528-36. Epub 2003 Dec 2. PubMed PMID: 14660584.
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Kidd JF, Kogan I, Bear CE. Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations. Curr Top Dev Biol. 2004;60:215-49. Review. PubMed PMID: 15094300.
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2003

Ramjeesingh M, Ugwu F, Li C, Dhani S, Huan LJ, Wang Y, Bear CE. Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore. Biochem J. 2003 Nov 1;375(Pt 3):633-41. PubMed PMID: 12892562; PubMed Central PMCID:
PMC1223717.
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Ramjeesingh M, Kidd JF, Huan LJ, Wang Y, Bear CE. Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane. Biochem J. 2003 Sep 15;374(Pt 3):793-7. PubMed PMID: 12820897; PubMed Central PMCID: PMC1223644.
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Mohammad-Panah R, Harrison R, Dhani S, Ackerley C, Huan LJ, Wang Y, Bear CE. The chloride channel ClC-4 contributes to endosomal acidification and trafficking. J Biol Chem. 2003 Aug 1;278(31):29267-77. Epub 2003 May 13. PubMed PMID: 12746443.
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Dhani SU, Mohammad-Panah R, Ahmed N, Ackerley C, Ramjeesingh M, Bear CE. Evidence for a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. J Biol Chem. 2003 May 2;278(18):16262-70. Epub 2003 Feb 23. PubMed PMID: 12601004.
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Kogan I, Ramjeesingh M, Li C, Kidd JF, Wang Y, Leslie EM, Cole SP, Bear CE. CFTR directly mediates nucleotide-regulated glutathione flux. EMBO J. 2003 May 1;22(9):1981-9. PubMed PMID: 12727866; PubMed Central PMCID: PMC156066.
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