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Items: 17


ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.

Wu YS, Jiang J, Ahmadi S, Lew A, Laselva O, Xia S, Bartlett C, Ip W, Wellhauser L, Ouyang H, Gonska T, Moraes TJ, Bear CE.

Mol Pharmacol. 2019 Oct;96(4):515-525. doi: 10.1124/mol.119.117143. Epub 2019 Aug 19.


Tumour necrosis factor α induces neuroinflammation and insulin resistance in immortalised hypothalamic neurones through independent pathways.

Clemenzi MN, Wellhauser L, Aljghami ME, Belsham DD.

J Neuroendocrinol. 2019 Jan;31(1):e12678. doi: 10.1111/jne.12678. Epub 2019 Jan 20.


The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.

Eckford PDW, McCormack J, Munsie L, He G, Stanojevic S, Pereira SL, Ho K, Avolio J, Bartlett C, Yang JY, Wong AP, Wellhauser L, Huan LJ, Jiang JX, Ouyang H, Du K, Klingel M, Kyriakopoulou L, Gonska T, Moraes TJ, Strug LJ, Rossant J, Ratjen F, Bear CE.

J Cyst Fibros. 2019 Jan;18(1):35-43. doi: 10.1016/j.jcf.2018.03.013. Epub 2018 Oct 10.


Orkambi® and amplifier co-therapy improves function from a rare CFTR mutation in gene-edited cells and patient tissue.

Molinski SV, Ahmadi S, Ip W, Ouyang H, Villella A, Miller JP, Lee PS, Kulleperuma K, Du K, Di Paola M, Eckford PD, Laselva O, Huan LJ, Wellhauser L, Li E, Ray PN, Pomès R, Moraes TJ, Gonska T, Ratjen F, Bear CE.

EMBO Mol Med. 2017 Sep;9(9):1224-1243. doi: 10.15252/emmm.201607137.


Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.

Ahmadi S, Bozoky Z, Di Paola M, Xia S, Li C, Wong AP, Wellhauser L, Molinski SV, Ip W, Ouyang H, Avolio J, Forman-Kay JD, Ratjen F, Hirota JA, Rommens J, Rossant J, Gonska T, Moraes TJ, Bear CE.

NPJ Genom Med. 2017 Apr 14;2:12. doi: 10.1038/s41525-017-0015-6.


High fat induces acute and chronic inflammation in the hypothalamus: effect of high-fat diet, palmitate and TNF-α on appetite-regulating NPY neurons.

Dalvi PS, Chalmers JA, Luo V, Han DY, Wellhauser L, Liu Y, Tran DQ, Castel J, Luquet S, Wheeler MB, Belsham DD.

Int J Obes (Lond). 2017 Jan;41(1):149-158. doi: 10.1038/ijo.2016.183. Epub 2016 Oct 24.


Nitric Oxide Exerts Basal and Insulin-Dependent Anorexigenic Actions in POMC Hypothalamic Neurons.

Wellhauser L, Chalmers JA, Belsham DD.

Mol Endocrinol. 2016 Apr;30(4):402-16. doi: 10.1210/me.2015-1275. Epub 2016 Mar 1.


Delineating the regulation of energy homeostasis using hypothalamic cell models.

Wellhauser L, Gojska NM, Belsham DD.

Front Neuroendocrinol. 2015 Jan;36:130-49. doi: 10.1016/j.yfrne.2014.09.001. Epub 2014 Sep 16. Review.


Activation of the omega-3 fatty acid receptor GPR120 mediates anti-inflammatory actions in immortalized hypothalamic neurons.

Wellhauser L, Belsham DD.

J Neuroinflammation. 2014 Mar 27;11:60. doi: 10.1186/1742-2094-11-60.


Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5.

D'Antonio C, Molinski S, Ahmadi S, Huan LJ, Wellhauser L, Bear CE.

Biochem J. 2013 Jun 15;452(3):391-400. doi: 10.1042/BJ20121848.


ATP induces conformational changes in the carboxyl-terminal region of ClC-5.

Wellhauser L, Luna-Chavez C, D'Antonio C, Tainer J, Bear CE.

J Biol Chem. 2011 Feb 25;286(8):6733-41. doi: 10.1074/jbc.M110.175877. Epub 2010 Dec 20.


A chemical corrector modifies the channel function of F508del-CFTR.

Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE.

Mol Pharmacol. 2010 Sep;78(3):411-8. doi: 10.1124/mol.110.065862. Epub 2010 May 25.


ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5.

Wellhauser L, D'Antonio C, Bear CE.

Pflugers Arch. 2010 Jul;460(2):543-57. doi: 10.1007/s00424-009-0769-5. Epub 2010 Jan 5. Review.


An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice.

Mohammad-Panah R, Wellhauser L, Steinberg BE, Wang Y, Huan LJ, Liu XD, Bear CE.

J Cell Sci. 2009 Apr 15;122(Pt 8):1229-37. doi: 10.1242/jcs.037317.


A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability.

Wellhauser L, Kim Chiaw P, Pasyk S, Li C, Ramjeesingh M, Bear CE.

Mol Pharmacol. 2009 Jun;75(6):1430-8. doi: 10.1124/mol.109.055608. Epub 2009 Apr 1.


Nucleotides bind to the C-terminus of ClC-5.

Wellhauser L, Kuo HH, Stratford FL, Ramjeesingh M, Huan LJ, Luong W, Li C, Deber CM, Bear CE.

Biochem J. 2006 Sep 1;398(2):289-94.


Developmental partitioning of myelin basic protein into membrane microdomains.

DeBruin LS, Haines JD, Wellhauser LA, Radeva G, Schonmann V, Bienzle D, Harauz G.

J Neurosci Res. 2005 Apr 15;80(2):211-25.


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