URMC / Labs / Thornton Lab / Publications Publications Wagner SDStruck AJGupta RFarnsworth DRMahady AEEichinger KThornton CAWang ETBerglund JA (2016 Sep). Dose-Dependent Regulation of Alternative Splicing by MBNL Proteins Reveals Biomarkers for Myotonic Dystrophy. PLoS genetics. Carrell STCarrell EMAuerbach DPandey SKBennett CFDirksen RTThornton CA (2016 Aug 13). Dmpk Gene Deletion or Antisense Knockdown Does Not Compromise Cardiac or Skeletal Muscle Function in Mice. Human molecular genetics. Burns TMSmith GAAllen JAAmato AAArnold WDBarohn RBenatar MBird SJBromberg MChahin NCiafaloni ECohen JACorse ACrum BADavid WSDimberg EDe Sousa EADonofrio PDDyck PJEngel AGEnsrud ERFerrante MFreimer MGable KLGibson SGilchrist JMGoldstein JMGooch CLGoodman BPGorelov DGospe SMGoyal NAGuidon ACGuptill JTGutmann LGutmann LGwathmey KHarati YHarper CMHehir MKHobson-Webb LDHoward JFJackson CEJohnson NJones SMJuel VCKaminski HJKaram CKennelly KDKhella SKhoury JKincaid JCKissel JTKolb NLacomis DLadha SLarriviere DLewis RALi YLitchy WJLogigian ELou JSMacGowen DJMaselli RMassey JMMauermann MLMathews KDMeriggioli MNMiller RGMoon JSMozaffar TNations SPNowak RJOstrow LWPascuzzi RMPeltier ARuzhansky KRichman DPRoss MARubin DIRussell JASachs GMSalajegheh MKSaperstein DSScelsa SSelcen DShaibani ASheih PBSilvestri NJSingleton JRSmith BESo YTSolorzano GSorenson EJSrinivasen JTavee JTawil RThaisetthawatkul PThornton CTrivedi JVernino SWang AKWebb TAWeiss MDWindebank AJWolfe GI (2015 Dec 10). Editorial by concerned physicians: Unintended effect of the Orphan Drug Act on the potential cost of 3,4-diaminopyridine. Muscle & nerve. Heatwole CJohnson NBode RDekdebrun JDilek NHilbert JELuebbe EMartens WMcDermott MPQuinn CRothrock NThornton CVickrey BGVictorson DMoxley RT (2015 Nov 18). Patient-Reported Impact of Symptoms in Myotonic Dystrophy Type 2 (PRISM-2). Neurology. Pandey SKWheeler TMJustice SLKim AYounis HSGattis DJauvin DPuymirat JSwayze EEFreier SMBennett CFThornton CAMacLeod AR (2015 Nov). Identification and Characterization of Modified Antisense Oligonucleotides Targeting DMPK in Mice and Nonhuman Primates for the Treatment of Myotonic Dystrophy Type 1. The Journal of pharmacology and experimental therapeutics. Heatwole CBode RNicholas Johnson Dekdebrun JDilek NEichinger KHilbert JELogigian ELuebbe EMartens WMcDermott MPPandya SPuwanant ARothrock NThornton CVickrey BGVictorson DMoxley RT (2015 Jun 4). The myotonic dystrophy health index: Correlations with clinical tests and patient function. Muscle & nerve. Wojtkowiak-Szlachcic ATaylor KStepniak-Konieczna ESznajder LJMykowska ASroka JThornton CASobczak K (2015 Mar 31). Short antisense-locked nucleic acids (all-LNAs) correct alternative splicing abnormalities in myotonic dystrophy. Nucleic acids research. Yadava RSFoff EPYu QGladman JTKim YKBhatt KSThornton CAZheng TSMahadevan MS (2014 Dec 11). TWEAK/Fn14, a pathway and novel therapeutic target in myotonic dystrophy. Human molecular genetics. Batra RCharizanis KManchanda MMohan ALi MFinn DJGoodwin MZhang CSobczak KThornton CASwanson MS (2014 Sep 24). Loss of MBNL Leads to Disruption of Developmentally Regulated Alternative Polyadenylation in RNA-Mediated Disease. Molecular cell. Thornton CA (2014 Aug). Myotonic Dystrophy. Neurologic clinics. Heatwole CBode RJohnson NDekdebrun JDilek NHeatwole MHilbert JELuebbe EMartens WMcdermott MPRothrock NThornton CVickrey BGVictorson DMoxley R (2014 Jun). Myotonic Dystrophy Health Index: initial evaluation of a disease-specific outcome measure. Muscle & nerve. Hoskins JWOfori LOChen CZKumar ASobczak KNakamori MSouthall NPatnaik SMarugan JJZheng WAustin CPDisney MDMiller BLThornton CA (2014 Jun 1). Lomofungin and dilomofungin: inhibitors of MBNL1-CUG RNA binding with distinct cellular effects. Nucleic acids research. Nakamori MSobczak KPuwanant AWelle SEichinger KPandya SDekdebrun JHeatwole CRMcDermott MPChen TCline MTawil ROsborne RJWheeler TMSwanson MSMoxley RTThornton CA (2013 Dec). Splicing biomarkers of disease severity in myotonic dystrophy. Annals of neurology. Axford MMWang YHNakamori MZannis-Hadjopoulos MThornton CAPearson CE (2013 Dec). Detection of slipped-DNAs at the trinucleotide repeats of the myotonic dystrophy type I disease locus in patient tissues. PLoS genetics. Coonrod LANakamori MWang WCarrell SHilton CLBodner MJSiboni RBDocter AGHaley MMThornton CABerglund JA (2013 Nov 15). Reducing levels of toxic RNA with small molecules. ACS chemical biology. Rzuczek SGGao YTang ZZThornton CAKodadek TDisney MD (2013 Oct 18). Features of modularly assembled compounds that impart bioactivity against an RNA target. ACS chemical biology. Sobczak KWheeler TMWang WThornton CA (2013 Feb). RNA interference targeting CUG repeats in a mouse model of myotonic dystrophy. Molecular therapy : the journal of the American Society of Gene Therapy. Childs-Disney JLStepniak-Konieczna ETran TYildirim IPark HChen CZHoskins JSouthall NMarugan JJPatnaik SZheng WAustin CPSchatz GCSobczak KThornton CADisney MD (2013). Induction and reversal of myotonic dystrophy type 1 pre-mRNA splicing defects by small molecules. Nature communications. Childs-Disney JLParkesh RNakamori MThornton CADisney MD (2012 Dec 21). Rational design of bioactive, modularly assembled aminoglycosides targeting the RNA that causes myotonic dystrophy type 1. ACS chemical biology. Wheeler TMLeger AJPandey SKMacLeod ARNakamori MCheng SHWentworth BMBennett CFThornton CA (2012 Aug 2). Targeting nuclear RNA for in vivo correction of myotonic dystrophy. Nature. Heatwole CBode RJohnson NQuinn CMartens WMcDermott MPRothrock NThornton CVickrey BVictorson DMoxley R (2012 Jul 24). Patient-reported impact of symptoms in myotonic dystrophy type 1 (PRISM-1). Neurology. Ofori LOHoskins JNakamori MThornton CAMiller BL (2012 Jul). From dynamic combinatorial 'hit' to lead: in vitro and in vivo activity of compounds targeting the pathogenic RNAs that cause myotonic dystrophy. Nucleic acids research. Childs-Disney JLHoskins JRzuczek SGThornton CADisney MD (2012 May 18). Rationally designed small molecules targeting the RNA that causes myotonic dystrophy type 1 are potently bioactive. ACS chemical biology. Tang ZZYarotskyy VWei LSobczak KNakamori MEichinger KMoxley RTDirksen RTThornton CA (2012 Mar 15). Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of Ca(V)1.1 calcium channel. Human molecular genetics. Parkesh RChilds-Disney JLNakamori MKumar AWang EWang THoskins JTran THousman DThornton CADisney MD (2012 Mar 14). Design of a bioactive small molecule that targets the myotonic dystrophy type 1 RNA via an RNA motif-ligand database and chemical similarity searching. Journal of the American Chemical Society. Hilbert JEKissel JTLuebbe EAMartens WBMcDermott MPSanders DBTawil RThornton CAMoxley RT (2012 Mar). If you build a rare disease registry, will they enroll and will they use it? Methods and data from the National Registry of Myotonic Dystrophy (DM) and Facioscapulohumeral Muscular Dystrophy (FSHD). Contemporary clinical trials. Chen CZSobczak KHoskins JSouthall NMarugan JJZheng WThornton CAAustin CP (2012 Feb). Two high-throughput screening assays for aberrant RNA-protein interactions in myotonic dystrophy type 1. Analytical and bioanalytical chemistry. Mankodi AWheeler TMShetty RSalceies KMBecher MWThornton CA (2012 Jan). Progressive myopathy in an inducible mouse model of oculopharyngeal muscular dystrophy. Neurobiology of disease. Nakamori MGourdon GThornton CA (2011 Dec). Stabilization of expanded (CTG)•(CAG) repeats by antisense oligonucleotides. Molecular therapy : the journal of the American Society of Gene Therapy. Axford MMLópez-Castel ANakamori MThornton CAPearson CE (2011 Jul). Replacement of the myotonic dystrophy type 1 CTG repeat with 'non-CTG repeat' insertions in specific tissues. Journal of medical genetics. López Castel ANakamori MThornton CAPearson CE (2011 Apr). Identification of restriction endonucleases sensitive to 5-cytosine methylation at non-CpG sites, including expanded (CAG)n/(CTG)n repeats. Epigenetics : official journal of the DNA Methylation Society. Nakamori MPearson CEThornton CA (2011 Feb 1). Bidirectional transcription stimulates expansion and contraction of expanded (CTG)*(CAG) repeats. Human molecular genetics. Heatwole CREichinger KJFriedman DIHilbert JEJackson CELogigian ELMartens WBMcDermott MPPandya SKQuinn CSmirnow AMThornton CAMoxley RT (2011 Jan). Open-label trial of recombinant human insulin-like growth factor 1/recombinant human insulin-like growth factor binding protein 3 in myotonic dystrophy type 1. Archives of neurology. López Castel ANakamori MTomé SChitayat DGourdon GThornton CAPearson CE (2011 Jan 1). Expanded CTG repeat demarcates a boundary for abnormal CpG methylation in myotonic dystrophy patient tissues. Human molecular genetics. Lueck JDRossi AEThornton CACampbell KPDirksen RT (2010 Dec). Sarcolemmal-restricted localization of functional ClC-1 channels in mouse skeletal muscle. The Journal of general physiology. Nakamori MThornton C (2010 Jul). Epigenetic changes and non-coding expanded repeats. Neurobiology of disease. Logigian ELMartens WBMoxley RTMcDermott MPDilek NWiegner AWPearson ATBarbieri CAAnnis CLThornton CAMoxley RT (2010 May 4). Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1. Neurology. Logigian ELTwydell PDilek NMartens WBQuinn CWiegner AWHeatwole CRThornton CAMoxley RT (2010 Feb). Evoked myotonia can be "dialed-up" by increasing stimulus train length in myotonic dystrophy type 1. Muscle & nerve. Du HCline MSOsborne RJTuttle DLClark TADonohue JPHall MPShiue LSwanson MSThornton CAAres M (2010 Feb). Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy. Nature structural & molecular biology. Lee MMChilds-Disney JLPushechnikov AFrench JMSobczak KThornton CADisney MD (2009 Dec 2). Controlling the specificity of modularly assembled small molecules for RNA via ligand module spacing: targeting the RNAs that cause myotonic muscular dystrophy. Journal of the American Chemical Society. Mueller CMHilbert JEMartens WThornton CAMoxley RTGreene MH (2009 Dec). Hypothesis: neoplasms in myotonic dystrophy. Cancer causes & control : CCC. Warf MBNakamori MMatthys CMThornton CABerglund JA (2009 Nov 3). Pentamidine reverses the splicing defects associated with myotonic dystrophy. Proceedings of the National Academy of Sciences of the United States of America. Nakamori MSobczak KMoxley RTThornton CA (2009 Nov). Scaled-down genetic analysis of myotonic dystrophy type 1 and type 2. Neuromuscular disorders : NMD. Welle SBurgess KThornton CATawil R (2009 Oct). Relation between extent of myostatin depletion and muscle growth in mature mice. American journal of physiology. Endocrinology and metabolism. Mulders SAvan den Broek WJWheeler TMCroes HJvan Kuik-Romeijn Pde Kimpe SJFurling DPlatenburg GJGourdon GThornton CAWieringa BWansink DG (2009 Aug 18). Triplet-repeat oligonucleotide-mediated reversal of RNA toxicity in myotonic dystrophy. Proceedings of the National Academy of Sciences of the United States of America. Pushechnikov ALee MMChilds-Disney JLSobczak KFrench JMThornton CADisney MD (2009 Jul 22). Rational design of ligands targeting triplet repeating transcripts that cause RNA dominant disease: application to myotonic muscular dystrophy type 1 and spinocerebellar ataxia type 3. Journal of the American Chemical Society. Wheeler TMSobczak KLueck JDOsborne RJLin XDirksen RTThornton CA (2009 Jul 17). Reversal of RNA dominance by displacement of protein sequestered on triplet repeat RNA. Science (New York, N.Y.). Osborne RJLin XWelle SSobczak KO'Rourke JRSwanson MSThornton CA (2009 Apr 15). Transcriptional and post-transcriptional impact of toxic RNA in myotonic dystrophy. Human molecular genetics. Gareiss PCSobczak KMcNaughton BRPalde PBThornton CAMiller BL (2008 Dec 3). Dynamic combinatorial selection of molecules capable of inhibiting the (CUG) repeat RNA-MBNL1 interaction in vitro: discovery of lead compounds targeting myotonic dystrophy (DM1). Journal of the American Chemical Society. Sorenson EJWindbank AJMandrekar JNBamlet WRAppel SHArmon CBarkhaus PEBosch PBoylan KDavid WSFeldman EGlass JGutmann LKatz JKing WLuciano CAMcCluskey LFNash SNewman DSPascuzzi RMPioro ESams LJScelsa SSimpson EPSubramony SHTiryaki EThornton CA (2008 Nov 25). Subcutaneous IGF-1 is not beneficial in 2-year ALS trial. Neurology. (2008 Aug 5). A trial of mycophenolate mofetil with prednisone as initial immunotherapy in myasthenia gravis. Neurology. Osborne RJThornton CA (2008 Mar). Cell-free cloning of highly expanded CTG repeats by amplification of dimerized expanded repeats. Nucleic acids research. Ciafaloni EMignot ESansone VHilbert JELin LLin XLiu LCPigeon WRPerlis MLThornton CA (2008 Jan 15). The hypocretin neurotransmission system in myotonic dystrophy type 1. Neurology. Yadava RSFrenzel-McCardell CDYu QSrinivasan VTucker ALPuymirat JThornton CAPrall OWHarvey RPMahadevan MS (2008 Jan). RNA toxicity in myotonic muscular dystrophy induces NKX2-5 expression. Nature genetics. Welle STawil RThornton CA (2008). Sex-related differences in gene expression in human skeletal muscle. PloS one. Wheeler TMLueck JDSwanson MSDirksen RTThornton CA (2007 Dec). Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophy. The Journal of clinical investigation. Wheeler TMThornton CA (2007 Oct). Myotonic dystrophy: RNA-mediated muscle disease. Current opinion in neurology. Moxley RTLogigian ELMartens WBAnnis CLPandya SMoxley RTBarbieri CADilek NWiegner AWThornton CA (2007 Sep). Computerized hand grip myometry reliably measures myotonia and muscle strength in myotonic dystrophy (DM1). Muscle & nerve. Lueck JDLungu CMankodi AOsborne RJWelle SLDirksen RTThornton CA (2007 Apr). Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1. American journal of physiology. Cell physiology. Welle SBhatt KPinkert CATawil RThornton CA (2007 Apr). Muscle growth after postdevelopmental myostatin gene knockout. American journal of physiology. Endocrinology and metabolism. Logigian ELCiafaloni EQuinn LCDilek NPandya SMoxley RTThornton CA (2007 Apr). Severity, type, and distribution of myotonic discharges are different in type 1 and type 2 myotonic dystrophy. Muscle & nerve. Wheeler TMKrym MCThornton CA (2007 Mar). Ribonuclear foci at the neuromuscular junction in myotonic dystrophy type 1. Neuromuscular disorders : NMD. Rudnicki DDHolmes SELin MWThornton CARoss CAMargolis RL (2007 Mar). Huntington's disease--like 2 is associated with CUG repeat-containing RNA foci. Annals of neurology. Osborne RJWelle SVenance SLThornton CATawil R (2007 Feb 20). Expression profile of FSHD supports a link between retinal vasculopathy and muscular dystrophy. Neurology. Lueck JDMankodi ASwanson MSThornton CADirksen RT (2007 Jan). Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy. The Journal of general physiology. Yuan YCompton SASobczak KStenberg MGThornton CAGriffith JDSwanson MS (2007). Muscleblind-like 1 interacts with RNA hairpins in splicing target and pathogenic RNAs. Nucleic acids research. Gwinn KCorriveau RAMitsumoto HBednarz KBrown RHCudkowicz MGordon PHHardy JKasarskis EJKaufmann PMiller RSorenson ETandan RTraynor BJNash JSherman AMailman MDOstell JBruijn LCwik VRich SSSingleton ARefolo LAndrews JZhang RConwit RKeller MA (2007). Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery. PloS one. Osborne RJThornton CA (2006 Oct 15). RNA-dominant diseases. Human molecular genetics. Kanadia RNShin JYuan YBeattie SGWheeler TMThornton CASwanson MS (2006 Aug 1). Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly(CUG) model for myotonic dystrophy. Proceedings of the National Academy of Sciences of the United States of America. Lin XMiller JWMankodi AKanadia RNYuan YMoxley RTSwanson MSThornton CA (2006 Jul 1). Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Human molecular genetics. Udd BMeola GKrahe RThornton CRanum LPBassez GKress WSchoser BMoxley R (2006 Jun). 140th ENMC International Workshop: Myotonic Dystrophy DM2/PROMM and other myotonic dystrophies with guidelines on management. Neuromuscular disorders : NMD. Mankodi ALin XBlaxall BCSwanson MSThornton CA (2005 Nov 25). Nuclear RNA foci in the heart in myotonic dystrophy. Circulation research. Gallo JMJin PThornton CALin HRobertson JD'Souza ISchlaepfer WW (2005 Nov 9). The role of RNA and RNA processing in neurodegeneration. The Journal of neuroscience : the official journal of the Society for Neuroscience. Logigian ELBlood CLDilek NMartens WBMoxley RTWiegner AWThornton CAMoxley RT (2005 Jul). Quantitative analysis of the "warm-up" phenomenon in myotonic dystrophy type 1. Muscle & nerve. Berg JJiang HThornton CACannon SC (2004 Dec 28). Truncated ClC-1 mRNA in myotonic dystrophy exerts a dominant-negative effect on the Cl current. Neurology. Jiang HMankodi ASwanson MSMoxley RTThornton CA (2004 Dec 15). Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons. Human molecular genetics. Logigian ELMoxley RTBlood CLBarbieri CAMartens WBWiegner AWThornton CAMoxley RT (2004 Apr 13). Leukocyte CTG repeat length correlates with severity of myotonia in myotonic dystrophy type 1. Neurology. Welle SBrooks AIDelehanty JMNeedler NBhatt KShah BThornton CA (2004 Mar). Skeletal muscle gene expression profiles in 20-29 year old and 65-71 year old women. Experimental gerontology. Kanadia RNJohnstone KAMankodi ALungu CThornton CAEsson DTimmers AMHauswirth WWSwanson MS (2003 Dec 12). A muscleblind knockout model for myotonic dystrophy. Science (New York, N.Y.). (2003 Dec 9). A comparison of muscle strength testing techniques in amyotrophic lateral sclerosis. Neurology. Mankodi ATeng-Umnuay PKrym MHenderson DSwanson MThornton CA (2003 Dec). Ribonuclear inclusions in skeletal muscle in myotonic dystrophy types 1 and 2. Annals of neurology. Bachinski LLUdd BMeola GSansone VBassez GEymard BThornton CAMoxley RTHarper PSRogers MTJurkat-Rott KLehmann-Horn FWieser TGamez JNavarro CBottani AKohler AShriver MDSallinen RWessman MZhang SWright FAKrahe R (2003 Oct). Confirmation of the type 2 myotonic dystrophy (CCTG)n expansion mutation in patients with proximal myotonic myopathy/proximal myotonic dystrophy of different European origins: a single shared haplotype indicates an ancestral founder effect. American journal of human genetics. Udd BMeola GKrahe RThornton CRanum LDay JBassez GRicker K (2003 Sep). Report of the 115th ENMC workshop: DM2/PROMM and other myotonic dystrophies. 3rd Workshop, 14-16 February 2003, Naarden, The Netherlands. Neuromuscular disorders : NMD. Welle SBrooks AIDelehanty JMNeedler NThornton CA (2003 Jul 7). Gene expression profile of aging in human muscle. Physiological genomics. Welle SBhatt KShah BNeedler NDelehanty JMThornton CA (2003 Apr). Reduced amount of mitochondrial DNA in aged human muscle. Journal of applied physiology (Bethesda, Md. : 1985). Thornton CAWelle SL (2002 Oct 22). Molecular fingerprints of inflammatory myopathies. Neurology. Mankodi AThornton CA (2002 Oct). Myotonic syndromes. Current opinion in neurology. Wolfe GIBarohn RJFoster BMJackson CEKissel JTDay JWThornton CANations SPBryan WWAmato AAFreimer MLParry GJ (2002 Oct). Randomized, controlled trial of intravenous immunoglobulin in myasthenia gravis. Muscle & nerve. Welle SBrooks AIThornton CA (2002 Aug 30). Computational method for reducing variance with Affymetrix microarrays. BMC bioinformatics. Mankodi ATakahashi MPJiang HBeck CLBowers WJMoxley RTCannon SCThornton CA (2002 Jul). Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy. Molecular cell. Welle SBhatt KShah BThornton C (2002 Jun). Insulin-like growth factor-1 and myostatin mRNA expression in muscle: comparison between 62-77 and 21-31 yr old men. Experimental gerontology. Meola GSansone VMarinou KCotelli MMoxley RTThornton CADe Ambroggi L (2002 Jan 15). Proximal myotonic myopathy: a syndrome with a favourable prognosis? Journal of the neurological sciences. Fougerousse FDurand MLopez SSuel LDemignon JThornton COzaki HKawakami KBarbet PBeckmann JSMaire P (2002). Six and Eya expression during human somitogenesis and MyoD gene family activation. Journal of muscle research and cell motility. Mankodi AUrbinati CRYuan QPMoxley RTSansone VKrym MHenderson DSchalling MSwanson MSThornton CA (2001 Sep 15). Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2. Human molecular genetics. Musser WSBarbano RLThornton CAMoxley RTHerrmann DNLogigian EL (2001 Sep). Distal myasthenia gravis with a decrement, an increment, and denervation. Journal of clinical neuromuscular disease. Rose MRMcDermott MPThornton CAPalenski CMartens WBGriggs RC (2001 Aug 14). A prospective natural history study of inclusion body myositis: implications for clinical trials. Neurology. Tapscott SJThornton CA (2001 Aug 3). Biomedicine. Reconstructing myotonic dystrophy. Science (New York, N.Y.). Welle SBrooks AThornton CA (2001 Mar 8). Senescence-related changes in gene expression in muscle: similarities and differences between mice and men. Physiological genomics. Mendell JRBarohn RJFreimer MLKissel JTKing WNagaraja HNRice RCampbell WWDonofrio PDJackson CELewis RAShy MSimpson DMParry GJRivner MHThornton CABromberg MBTandan RHarati YGiuliani MJ (2001 Feb 27). Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. Mankodi ALogigian ECallahan LMcClain CWhite RHenderson DKrym MThornton CA (2000 Sep 8). Myotonic dystrophy in transgenic mice expressing an expanded CUG repeat. Science (New York, N.Y.). Miller JWUrbinati CRTeng-Umnuay PStenberg MGByrne BJThornton CASwanson MS (2000 Sep 1). Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy. The EMBO journal. Welle SBhatt KThornton CA (2000 Jul). High-abundance mRNAs in human muscle: comparison between young and old. Journal of applied physiology (Bethesda, Md. : 1985). Forbes GBGriggs RCMoxley RTThornton CTTawil R (2000 May). K-40 and dual-energy X-ray absorptiometry estimates of lean weight compared. Normals and patients with neuromuscular disease. Annals of the New York Academy of Sciences. Tian BWhite RJXia TWelle STurner DHMathews MBThornton CA (2000 Jan). Expanded CUG repeat RNAs form hairpins that activate the double-stranded RNA-dependent protein kinase PKR. RNA (New York, N.Y.). Wax PMThornton CA (2000). Recovery from severe arsenic-induced peripheral neuropathy with 2,3-dimercapto-1-propanesulphonic acid. Journal of toxicology. Clinical toxicology. Meola GSansone VPerani DColleluori ACappa SCotelli MFazio FThornton CAMoxley RT (1999 Sep 22). Reduced cerebral blood flow and impaired visual-spatial function in proximal myotonic myopathy. Neurology. Schwid SRThornton CAPandya SManzur KLSanjak MPetrie MDMcDermott MPGoodman AD (1999 Sep 11). Quantitative assessment of motor fatigue and strength in MS. Neurology. Welle SBhatt KThornton CA (1999 May). Inventory of high-abundance mRNAs in skeletal muscle of normal men. Genome research. (1999 Apr 22). A controlled trial of recombinant methionyl human BDNF in ALS: The BDNF Study Group (Phase III). Neurology. Welle SBhatt KThornton CA (1999 Apr). Stimulation of myofibrillar synthesis by exercise is mediated by more efficient translation of mRNA. Journal of applied physiology (Bethesda, Md. : 1985). Mezei MMMankodi ABrais BMarineau CThornton CARouleau GAKarpati G (1999 Feb). Minimal expansion of the GCG repeat in the PABP2 gene does not predispose to sporadic inclusion body myositis. Neurology. Thornton CAAshizawa T (1999 Jan 1). Getting a grip on the myotonic dystrophies. Neurology. Thornton C (1999). The myotonic dystrophies. Seminars in neurology. Simmons ZThornton CASeltzer WKRichards CS (1998 May). Relative stability of a minimal CTG repeat expansion in a large kindred with myotonic dystrophy. Neurology. Welle SThornton CA (1998 Apr). High-protein meals do not enhance myofibrillar synthesis after resistance exercise in 62- to 75-yr-old men and women. The American journal of physiology. Welle SThornton C (1997 Oct). Insulin-like growth factor-I, actin, and myosin heavy chain messenger RNAs in skeletal muscle after an injection of growth hormone in subjects over 60 years old. The Journal of endocrinology. Welle SThornton CBhatt KKrym M (1997 Sep). Expression of elongation factor-1 alpha and S1 in young and old human skeletal muscle. The journals of gerontology. Series A, Biological sciences and medical sciences. Thornton CAWymer JPSimmons ZMcClain CMoxley RT (1997 Aug). Expansion of the myotonic dystrophy CTG repeat reduces expression of the flanking DMAHP gene. Nature genetics. Welle STotterman SThornton C (1996 Nov). Effect of age on muscle hypertrophy induced by resistance training. The journals of gerontology. Series A, Biological sciences and medical sciences. Welle SThornton CStatt MMcHenry B (1996 Sep). Growth hormone increases muscle mass and strength but does not rejuvenate myofibrillar protein synthesis in healthy subjects over 60 years old. The Journal of clinical endocrinology and metabolism. Thornton CAWelle SGriggs RCAbraham GN (1996 Jul 15). Human IgG production in vivo: determination of synthetic rate by nonradioactive tracer incorporation. Journal of immunology (Baltimore, Md. : 1950). Welle SThornton CTotterman SForbes G (1996 Feb). Utility of creatinine excretion in body-composition studies of healthy men and women older than 60 y. The American journal of clinical nutrition. Welle SBhatt KThornton C (1996 Feb). Polyadenylated RNA, actin mRNA, and myosin heavy chain mRNA in young and old human skeletal muscle. The American journal of physiology. Thornton CGriggs RCMoxley RT (1995 Aug). PROMM syndrome (Ricker's disease) Annals of neurology. Welle SThornton CStatt M (1995 Mar). Myofibrillar protein synthesis in young and old human subjects after three months of resistance training. The American journal of physiology. Rifai ZWelle SKamp CThornton CA (1995 Jan). Ragged red fibers in normal aging and inflammatory myopathy. Annals of neurology. Welle SThornton CStatt MMcHenry B (1994 Oct). Postprandial myofibrillar and whole body protein synthesis in young and old human subjects. The American journal of physiology. Thornton CAGriggs RC (1994 Mar). Plasma exchange and intravenous immunoglobulin treatment of neuromuscular disease. Annals of neurology. Thornton CAGriggs RCMoxley RT (1994 Mar). Myotonic dystrophy with no trinucleotide repeat expansion. Annals of neurology. Houston SThornton CEmmanuel JLatif A (1994 Mar). Human T cell lymphotropic virus type 1 in Zimbabwe. Transactions of the Royal Society of Tropical Medicine and Hygiene. Thornton CAJohnson KMoxley RT (1994 Jan). 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