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Nicholas E. Johnson

University of Georgia · US
Area of research
Cellular and Molecular Neuroscience · Molecular Biology
Research interest
Research interests include Genetic Neurodegenerative Diseases, Muscle Physiology and Disorders, Neurogenetic and Muscular Disorders Research, and Biochemical and Molecular Research.
h-index
34
citations
3,554
works
196
NIH funding
primary concept
email

Recent publications

Current clinical applications of AAV-mediated gene therapy
Molecular Therapy 2025cited by 59position: middledoi
The Splice Index as a prognostic biomarker of strength and function in myotonic dystrophy type 1
Journal of Clinical Investigation 2025cited by 15position: middledoi
Neurobehavioral Phenotype of Children With Congenital Myotonic Dystrophy
Neurology 2024cited by 9position: lastdoi
Antisense oligonucleotide targeting DMPK in patients with myotonic dystrophy type 1: a multicentre, randomised, dose-escalation, placebo-controlled, phase 1/2a trial
The Lancet Neurology 2023cited by 52position: middledoi
PCR87 Impact of Signs and Symptoms of Chronic Refractory Gout on Patient Health-Related Quality of Life
Value in Health 2023cited by 0position: middledoi
Randomized phase 2 study of<scp>ACE</scp>‐083, a<scp>muscle‐promoting</scp>agent, in facioscapulohumeral muscular dystrophy
Muscle & Nerve 2022cited by 25position: middledoi
Evaluation of effects of continued corticosteroid treatment on cardiac and pulmonary function in non‐ambulatory males with Duchenne muscular dystrophy from <scp>MD STAR<i>net</i></scp>
Muscle & Nerve 2022cited by 16position: middledoi
Population-Based Prevalence of Myotonic Dystrophy Type 1 Using Genetic Analysis of Statewide Blood Screening Program
Neurology 2021cited by 162position: firstdoi
p53 convergently activates Dux/DUX4 in embryonic stem cells and in facioscapulohumeral muscular dystrophy cell models
Nature Genetics 2021cited by 113position: middledoi
Reldesemtiv in Patients with Spinal Muscular Atrophy: a Phase 2 Hypothesis-Generating Study
Neurotherapeutics 2021cited by 44position: middledoi
Natural history of Charcot-Marie-Tooth disease type 2A: a large international multicentre study
Brain 2020cited by 89position: middledoi
Association of genetic mutations and loss of ambulation in childhood‐onset dystrophinopathy
Muscle & Nerve 2020cited by 20position: middledoi
Clinical trial readiness to solve barriers to drug development in FSHD (ReSolve): protocol of a large, international, multi-center prospective study
BMC Neurology 2019cited by 55position: middledoi
Limb‐girdle muscular dystrophy: A perspective from adult patients on what matters most
Muscle & Nerve 2019cited by 25position: lastdoi
Patient Reported Impact of Symptoms in Spinal Muscular Atrophy (PRISM-SMA)
Neurology 2018cited by 98position: middledoi
The <scp>C</scp>harcot–<scp>M</scp>arie–<scp>T</scp>ooth <scp>H</scp>ealth <scp>I</scp>ndex: Evaluation of a Patient‐Reported Outcome
Annals of Neurology 2018cited by 38position: firstdoi
Orofacial strength, dysarthria, and dysphagia in congenital myotonic dystrophy
Muscle & Nerve 2018cited by 35position: lastdoi
Myotonic dystrophy patient preferences in patient‐reported outcome measures
Muscle & Nerve 2018cited by 17position: middledoi
Review of the Diagnosis and Treatment of Periodic Paralysis
Muscle & Nerve 2017cited by 261position: middledoi
Patient-Centered Therapy Development for Myotonic Dystrophy: Report of the Myotonic Dystrophy Foundation–Sponsored Workshop
Therapeutic Innovation & Regulatory Science 2017cited by 4position: middledoi
Management of Charcot&amp;ndash;Marie&amp;ndash;Tooth disease: improving long-term care with a multidisciplinary approach
Journal of Multidisciplinary Healthcare 2016cited by 47position: firstdoi
A population-based survey of risk for cancer in individuals diagnosed with myotonic dystrophy
Muscle & Nerve 2016cited by 32position: middledoi
The Impact of Pregnancy on Myotonic Dystrophy: A Registry-Based Study
Journal of Neuromuscular Diseases 2015cited by 60position: firstdoi
Patient-Reported Impact of Symptoms in Myotonic Dystrophy Type 2 (PRISM-2)
Neurology 2015cited by 59position: middledoi
Parent‐reported multi‐national study of the impact of congenital and childhood onset myotonic dystrophy
Developmental Medicine & Child Neurology 2015cited by 52position: firstdoi
Myotonic dystrophy health index: Correlations with clinical tests and patient function
Muscle & Nerve 2015cited by 52position: middledoi
Quality-of-life in Charcot–Marie–Tooth disease: The patient’s perspective
Neuromuscular Disorders 2014cited by 108position: firstdoi
Myotonic Dystrophy Health Index: Initial evaluation of a disease‐specific outcome measure
Muscle & Nerve 2013cited by 62position: middledoi

Grants

NSF East Asia and Pacific Summer Institute (EAPSI) for FY 2013 in Australia
NSF1311040$5,0702013–2014PIRePORTER

Frequent collaborators

Chad Heatwole · Bangladesh University of Engineering and Technology12 papers (2013–2023)Charles A. Thornton · University of Rochester Medical Center7 papers (2013–2025)Michael McDermott · Luzerner Kantonsspital7 papers (2013–2019)Russell J. Butterfield · University of Utah7 papers (2018–2024)Richard T. Moxley · University of Rochester7 papers (2013–2023)Nuran Dilek · University of Rochester7 papers (2013–2018)Man Hung · Roseman University of Health Sciences6 papers (2015–2025)Elizabeth Luebbe · University of Rochester6 papers (2013–2018)Jeanne Dekdebrun · University of Rochester Medical Center5 papers (2013–2025)Jeffrey Statland · George Washington University5 papers (2017–2023)James E. Hilbert · University of Rochester5 papers (2013–2018)William B. Martens · University of Rochester Medical Center4 papers (2013–2018)Kiera N. Berggren · Virginia Commonwealth University3 papers (2018–2025)Nan Rothrock · Northwestern University3 papers (2013–2015)Barbara G. Vickrey · Hudson Institute3 papers (2013–2015)Katy Eichinger · University of Rochester Medical Center3 papers (2015–2023)Rita Bode · Trent University3 papers (2013–2015)Perry B. Shieh · Veterans Affairs Canada3 papers (2017–2021)David Victorson · Northwestern University3 papers (2013–2015)John T. Kissel · The Ohio State University Wexner Medical Center3 papers (2017–2023)