C
Christopher R. Heier
Researcher at George Washington University
Publications - 33
Citations - 1297
Christopher R. Heier is an academic researcher from George Washington University. The author has contributed to research in topics: Duchenne muscular dystrophy & Muscular dystrophy. The author has an hindex of 18, co-authored 25 publications receiving 1095 citations. Previous affiliations of Christopher R. Heier include Indiana University & National Institutes of Health.
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Journal ArticleDOI
Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction.
Rocky G. Gogliotti,Katharina A. Quinlan,Courtenay B. Barlow,Christopher R. Heier,Charles J. Heckman,Christine J. DiDonato +5 more
TL;DR: It is demonstrated that the SMA phenotype autonomously originates in MNs and that sensory-motor synapse loss is a consequence, not a cause, of MN dysfunction.
Journal ArticleDOI
Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice
TL;DR: It is found SMA mice suffer from severe bradyarrhythmia characterized by progressive heart block and impaired ventricular depolarization, and a model in which decreased sympathetic innervation causes autonomic imbalance is proposed, characterized by a relative increase in the level of vagal tone controlling heart rate.
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VBP15, a novel anti-inflammatory and membrane-stabilizer, improves muscular dystrophy without side effects
Christopher R. Heier,Jesse M. Damsker,Qing Yu,Blythe C. Dillingham,Tony Huynh,Jack H. Van der Meulen,Arpana Sali,Brittany K. Miller,Aditi Phadke,Luana Scheffer,James L Quinn,Kathleen Tatem,Sarah Jordan,Sherry Dadgar,Olga Rodriguez,Chris Albanese,Michael E. Calhoun,Heather Gordish-Dressman,Jyoti K. Jaiswal,Edward M. Connor,John M. McCall,Eric P. Hoffman,Erica K.M. Reeves,Kanneboyina Nagaraju +23 more
TL;DR: Successful improvement of dystrophy independent of hormonal, growth, or immunosuppressive effects is demonstrated, indicating VBP15 merits clinical investigation for DMD and would benefit other chronic inflammatory diseases.
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Translational readthrough by the aminoglycoside geneticin (G418) modulates SMN stability in vitro and improves motor function in SMA mice in vivo
TL;DR: Stable cell lines are used to demonstrate the SMN C-terminus modulates protein stability in a sequence-independent manner that is reproducible by translational readthrough and provide the first in vivo evidence supporting translationalreadthrough as a therapeutic strategy for the treatment of SMA.
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The DcpS inhibitor RG3039 improves survival, function and motor unit pathologies in two SMA mouse models
Rocky G. Gogliotti,Herminio J. Cardona,Jasbir Singh,Sophie Bail,Carina Emery,Carina Emery,Nancy L. Kuntz,Nancy L. Kuntz,Michael Jorgensen,Madel Durens,Bing Xia,Courtenay B. Barlow,Courtenay B. Barlow,Christopher R. Heier,Christopher R. Heier,Heather L. Plasterer,Vincent Jacques,Megerditch Kiledjian,Jill Jarecki,James R. Rusche,Christine J. DiDonato,Christine J. DiDonato +21 more
TL;DR: The minimum effective dose and the associated pharmacokinetic (PK) and exposure relationship of RG3039 and DcpS inhibition ex vivo are determined and support the long PK half-life with extended pharmacodynamic outcome ofRG3039 in 2B/- SMA mice.