URMC / Labs / Lueck Lab / Publications Recent Publications Franchini LPorter JJLueck JDOrlandi C Gz Enhanced Signal Transduction assaY (G Z ESTY) for GPCR deorphanization.; bioRxiv : the preprint server for biology. 2024 Jul 26. Porter JJLueck JD A cystic fibrosis gene editing approach that is on target.; Molecular therapy. Nucleic acids; Vol 35(2), pp. 102197. 2024 May 08. Cisco LASipple MTEdwards KMThornton CALueck JD Verapamil mitigates chloride and calcium bi-channelopathy in a myotonic dystrophy mouse model.; The Journal of clinical investigation; Vol 134(1). 2024 Jan 02. Cisco LASipple MTEdwards KMThornton CALueck JD Combinatorial chloride and calcium channelopathy in myotonic dystrophy.; bioRxiv : the preprint server for biology. 2023 Jun 01. Allaire NEGriesenbach UKerem BLueck JDStanleigh NOren YS Gene, RNA, and ASO-based therapeutic approaches in Cystic Fibrosis.; Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2023 Jan 17. Rook MLMcCullock TWCouch TLueck JDMacLean DM Photomodulation of the ASIC1a acidic pocket destabilizes the open state.; Protein science : a publication of the Protein Society. 2023 Jan 08. Ko WPorter JJSipple MTEdwards KMLueck JD Efficient suppression of endogenous CFTR nonsense mutations using anticodon-engineered transfer RNAs.; Molecular therapy. Nucleic acids; Vol 28. 2022 Jun 14. Porter JJHeil CSLueck JD Therapeutic promise of engineered nonsense suppressor tRNAs.; Wiley interdisciplinary reviews. RNA. 2021 Feb 10. Rook MLWilliamson ALueck JDMusgaard MMaclean DM β11-12 linker isomerization governs acid-sensing ion channel desensitization and recovery.; eLife; Vol 9. 2020 Feb 07. Lueck JDYoon JSPerales-Puchalt AMackey ALInfield DTBehlke MAPope MRWeiner DBSkach WRMcCray PBAhern CA Engineered transfer RNAs for suppression of premature termination codons.; Nature communications; Vol 10(1). 2019 Feb 18. Molinarolo SLee SLeisle LLueck JDGranata DCarnevale VAhern CA Cross-kingdom auxiliary subunit modulation of a voltage-gated sodium channel.; The Journal of biological chemistry; Vol 293(14). 2018 Apr 06. Infield DTLueck JDGalpin JDGalles GDAhern CA Orthogonality of Pyrrolysine tRNA in the Xenopus oocyte.; Scientific reports; Vol 8(1). 2018 Mar 26. Leisle LChadda RLueck JDInfield DTGalpin JDKrishnamani VRobertson JLAhern CA Cellular encoding of Cy dyes for single-molecule imaging.; eLife; Vol 5. 2016 Jan 12. Lueck JDMackey ALInfield DTGalpin JDLi JRoux BAhern CA Atomic mutagenesis in ion channels with engineered stoichiometry.; eLife; Vol 5. 2016 Jan 06. Beedle AMTurner AJSaito YLueck JDFoltz SJFortunato MJNienaber PMCampbell KP Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy.; The Journal of clinical investigation; Vol 122(9). 2012 Sep. Loy RELueck JDMostajo-Radji MACarrell EMDirksen RT Allele-specific gene silencing in two mouse models of autosomal dominant skeletal myopathy.; PloS one; Vol 7(11). 2012. Han RFrett EMLevy JRRader EPLueck JDBansal DMoore SANg RBeltrán-Valero de Bernabé DFaulkner JACampbell KP Genetic ablation of complement C3 attenuates muscle pathology in dysferlin-deficient mice.; The Journal of clinical investigation; Vol 120(12). 2010 Dec. Lueck JDRossi AEThornton CACampbell KPDirksen RT Sarcolemmal-restricted localization of functional ClC-1 channels in mouse skeletal muscle.; The Journal of general physiology; Vol 136(6). 2010 Dec. Wheeler TMSobczak KLueck JDOsborne RJLin XDirksen RTThornton CA Reversal of RNA dominance by displacement of protein sequestered on triplet repeat RNA.; Science (New York, N.Y.); Vol 325(5938). 2009 Jul 17. Kimura TLueck JDHarvey PJPace SMIkemoto NCasarotto MGDirksen RTDulhunty AF Alternative splicing of RyR1 alters the efficacy of skeletal EC coupling.; Cell calcium; Vol 45(3). 2009 Mar. Wheeler TMLueck JDSwanson MSDirksen RTThornton CA Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophy.; The Journal of clinical investigation; Vol 117(12). 2007 Dec. Lueck JDLungu CMankodi AOsborne RJWelle SLDirksen RTThornton CA Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1.; American journal of physiology. Cell physiology; Vol 292(4). 2007 Apr. Lueck JDMankodi ASwanson MSThornton CADirksen RT Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.; The Journal of general physiology; Vol 129(1). 2007 Jan. Chelu MGGoonasekera SADurham WJTang WLueck JDRiehl JPessah INZhang PBhattacharjee MBDirksen RTHamilton SL Heat- and anesthesia-induced malignant hyperthermia in an RyR1 knock-in mouse.; FASEB journal : official publication of the Federation of American Societies for Experimental Biology; Vol 20(2). 2006 Feb. Kimura TNakamori MLueck JDPouliquin PAoike FFujimura HDirksen RTTakahashi MPDulhunty AFSakoda S Altered mRNA splicing of the skeletal muscle ryanodine receptor and sarcoplasmic/endoplasmic reticulum Ca2+-ATPase in myotonic dystrophy type 1.; Human molecular genetics; Vol 14(15). 2005 Aug 1.