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Barrington G. Burnett · SciRah

Researcher profile · Semantic Scholar

Barrington G. Burnett

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Barrington G. Burnett — researcher profile on SciRah

h-index: 26
Works: 54
Citations: 3,963

Most-cited papers

Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice
L. Kong, Xue-Yong Wang, Dong W. Choe, Michelle Polley +5 more
2009Journal of Neuroscience399 citations Open access
Semantic Scholar
The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity.
Barrington G. Burnett, Fusheng Li, R. Pittman
2003Human Molecular Genetics380 citations Open access
Semantic Scholar
Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy.
Amy M. Avila, Barrington G. Burnett, A. Taye, F. Gabanella +7 more
2007Journal of Clinical Investigation344 citations Open access
Semantic Scholar
Mutations in TRPV4 cause Charcot-Marie-Tooth disease type 2C
G. Landouré, A. Zdebik, Tara L. Martinez, Barrington G. Burnett +16 more
2009Nature Genetics317 citations Open access
Semantic Scholar
Enabling the genomic revolution in Africa
C. Rotimi, Akin Abayomi, A. Abimiku, V. Adabayeri +241 more
2014Science305 citations Open access
Semantic Scholar
Regulation of SMN Protein Stability
Barrington G. Burnett, E. Muñoz, Animesh Tandon, Deborah Y. Kwon +2 more
2008Molecular and Cellular Biology279 citations Open access
Semantic Scholar
MicroRNA pathways modulate polyglutamine-induced neurodegeneration.
Julide Bilen, Nan Liu, Barrington G. Burnett, R. Pittman +1 more
2006Molecules and Cells250 citations Open access
Semantic Scholar
The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation.
Barrington G. Burnett, R. Pittman
2005Proceedings of the National Academy of Sciences of the United States of America180 citations Open access
Semantic Scholar
Sustained Improvement of Spinal Muscular Atrophy Mice Treated with Trichostatin A Plus Nutrition
H. Narver, L. Kong, Barrington G. Burnett, Dong W. Choe +4 more
2008Annals of Neurology162 citations Open access
Semantic Scholar
Mitochondrial abnormalities in spinal and bulbar muscular atrophy
Srikanth Ranganathan, G. G. Harmison, K. Meyertholen, M. Pennuto +2 more
2008Human Molecular Genetics160 citations Open access
Semantic Scholar
Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity.
Isabella Palazzolo, Barrington G. Burnett, Jessica E. Young, Phebe L Brenne +4 more
2007Human Molecular Genetics157 citations Open access
Semantic Scholar
Cowchock syndrome is associated with a mutation in apoptosis-inducing factor.
C. Rinaldi, C. Grunseich, I. Sevrioukova, Alice B. Schindler +10 more
2012American Journal of Human Genetics142 citations Open access
Semantic Scholar
Hereditary spastic paraplegia type 43 (SPG43) is caused by mutation in C19orf12
G. Landouré, Peng-Peng Zhu, C. Lourenço, Janel O. Johnson +27 more
2013Human Mutation85 citations Open access
Semantic Scholar
Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics.
Katherine V. Bricceno, Tara L. Martinez, E. Leikina, S. Duguez +5 more
2014Human Molecular Genetics79 citations Open access
Semantic Scholar
Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice.
Deborah Y. Kwon, W. Motley, K. Fischbeck, Barrington G. Burnett
2011Human Molecular Genetics74 citations Open access
Semantic Scholar
Histone deacetylase inhibition suppresses myogenin-dependent atrogene activation in spinal muscular atrophy mice.
Katherine V. Bricceno, Paul J. Sampognaro, James P. Van Meerbeke, C. Sumner +2 more
2012Human Molecular Genetics70 citations Open access
Semantic Scholar
The E3 ubiquitin ligase mind bomb 1 ubiquitinates and promotes the degradation of survival of motor neuron protein
Deborah Y. Kwon, Maria Dimitriadi, B. Terzic, C. Cable +4 more
2013Molecular Biology of the Cell61 citations Open access
Semantic Scholar
Non-targeted Identification of Prions and Amyloid-forming Proteins from Yeast and Mammalian Cells*
D. Kryndushkin, N. Pripuzova, Barrington G. Burnett, F. Shewmaker
2013Journal of Biological Chemistry58 citations Open access
Semantic Scholar
Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics.
Barrington G. Burnett, J. Andrews, Srikanth Ranganathan, K. Fischbeck +1 more
2008Neurobiology of Disease51 citations Open access
Semantic Scholar
The role of the immunoproteasome in interferon-γ-mediated microglial activation
Kasey E. Moritz, Nikki M. McCormack, Mahlet B. Abera, C. Viollet +4 more
2017Scientific Reports48 citations Open access
Semantic Scholar
Genetics of low spinal muscular atrophy carrier frequency in sub‐Saharan Africa
M. Sangare, B. Hendrickson, Hammadoun A Sango, Ke-Lian Chen +32 more
2014Annals of Neurology40 citations Open access
Semantic Scholar
CNS uptake of bortezomib is enhanced by P-glycoprotein inhibition: implications for spinal muscular atrophy
E. Foran, Deborah Y. Kwon, J. Nofziger, Eveline S. Arnold +3 more
2016Neurobiology of Disease35 citations Open access
Semantic Scholar
MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy
Naemeh Pourshafie, Philip R. Lee, Ke-Lian Chen, G. G. Harmison +6 more
2016Molecular Therapy33 citations Open access
Semantic Scholar
Exome sequencing identifies a novel TRPV4 mutation in a CMT2C family
G. Landouré, Jeremy M. Sullivan, Janel O. Johnson, C. Munns +9 more
2012Neurology33 citations Open access
Semantic Scholar
Emerging treatment options for spinal muscular atrophy
Barrington G. Burnett, T. Crawford, C. Sumner
2009Current Treatment Options in Neurology31 citations
Semantic Scholar
Viral mediated knockdown of GATA6 in SMA iPSC‐derived astrocytes prevents motor neuron loss and microglial activation
R. Allison, Emily Welby, Guzal Khayrullina, Barrington G. Burnett +1 more
2021bioRxiv27 citations Open access
Semantic Scholar
A candidate gene for autoimmune myasthenia gravis
G. Landouré, M. A. Knight, H. Stanescu, A. Taye +13 more
2012Neurology23 citations Open access
Semantic Scholar
SMN-deficiency disrupts SERCA2 expression and intracellular Ca2+ signaling in cardiomyocytes from SMA mice and patient-derived iPSCs
Guzal Khayrullina, Kasey E. Moritz, J. Schooley, N. Fatima +7 more
2020Skeletal Muscle21 citations Open access
Semantic Scholar
ML372 blocks SMN ubiquitination and improves spinal muscular atrophy pathology in mice.
Mahlet B. Abera, Jing-Bo Xiao, J. Nofziger, Steve Titus +12 more
2016JCI Insight21 citations Open access
Semantic Scholar
Discovery of a Small Molecule Probe That Post-Translationally Stabilizes the Survival Motor Neuron Protein for the Treatment of Spinal Muscular Atrophy
A. Rietz, Hong-Xia Li, Kevin M. Quist, J. Cherry +13 more
2017Journal of Medicinal Chemistry16 citations Open access
Semantic Scholar
Survival motor neuron protein deficiency alters microglia reactivity
Guzal Khayrullina, Zaida A Alipio-Gloria, M. Deguise, Sabrina Gagnon +10 more
2022Glia15 citations Open access
Semantic Scholar
Survival motor neuron deficiency slows myoblast fusion through reduced myomaker and myomixer expression
Nikki M. McCormack, Eric Villalón, C. Viollet, A. Soltis +3 more
2021Journal of Cachexia, Sarcopenia and Muscle14 citations Open access
Semantic Scholar
A high-throughput genome-wide RNAi screen identifies modifiers of survival motor neuron protein
Nikki M. McCormack, Mahlet B. Abera, Eveline S. Arnold, Rebecca M. Gibbs +6 more
2021Cell Reports13 citations Open access
Semantic Scholar
Heterozygous UBR5 variants result in a neurodevelopmental syndrome with developmental delay, autism, and intellectual disability.
Pascale Sabeh, Samantha A. Dumas, C. Maios, Hiba Daghar +65 more
2024American Journal of Human Genetics10 citations Open access
Semantic Scholar
Genetics and genomic medicine in Mali: challenges and future perspectives
G. Landouré, O. Samassékou, M. Traoré, K. Meilleur +4 more
2016Molecular Genetics & Genomic Medicine9 citations Open access
Semantic Scholar
Neurogenic and Myogenic Contributions to Hereditary Motor Neuron Disease
Katherine V. Bricceno, K. Fischbeck, Barrington G. Burnett
2012Neurodegenerative Diseases7 citations Open access
Semantic Scholar
Targeting splicing in spinal muscular atrophy
Barrington G. Burnett, Charlotte J. Sumner
2008Annals of Neurology3 citations
Semantic Scholar
The Ubiquitin E3 Ligase UBE3A Regulates GRIPAP1 and PACSIN1 Proteins Linked to the Endocytic Recycling of AMPA Receptors
Laura Drebushenko, Natalya Belous, F. Lischka, Qiong Zhou +4 more
2025Molecular and Cellular Biology2 citations Open access
Semantic Scholar
A combinatorial approach increases SMN level in SMA model mice.
Samantha A. Dumas, Eric Villalón, Elizabeth M. Bergman, K. J. Wilson +3 more
2022Human Molecular Genetics2 citations Open access
Semantic Scholar
Does the survival motor neuron copy number variation play a role in the onset and severity of sporadic amyotrophic lateral sclerosis in Malians?
M. Sangare, I. Dicko, C. Guinto, A. Sissoko +29 more
2016eNeurologicalSci2 citations Open access
Semantic Scholar
SMN Modulator ML372 Increases SMN Protein Abundance, Body Weight, Lifespan, and Rescues Motor Function in SMNΔ7 SMA Mice
Barrington G. Burnett, Jing-Bo Xiao, Noel Southall, Wei Zheng +5 more
20152 citations
Semantic Scholar
Transcriptional reprogramming in SMA mouse hearts reveals signatures of early heart failure and dysregulated calcium signaling.
Cecelia C. Mangione, A. Frank, C. Dalgard, Barrington G. Burnett +1 more
2025Human Molecular Genetics1 citations Open access
Semantic Scholar
Key features of the innate immune response is mediated by the immunoproteasome in microglia
Salman Izadjoo, Kasey E. Moritz, Guzal Khayrullina, Elizabeth M. Bergman +8 more
2025Scientific Reports1 citations Open access
Semantic Scholar
Advancing neurogenetics in Africa: past achievements, current developments and shaping the future
G. Landouré, A. Yalcouyé, S. Diarra, A. Maiga +8 more
2025Nature Reviews Neurology1 citations
Semantic Scholar
IL-1ra and CCL5, but not IL-10, are promising targets for treating SMA astrocyte-driven pathology
R. Allison, Cecelia C. Mangione, Mya Suneja, Jessica E. Gawrys +6 more
2024Molecular Therapy1 citations Open access
Semantic Scholar
The role of the immunoproteasome in interferon-γ-mediated microglial activation
Kasey E. Moritz, Nikki M. McCormack, Mahlet B. Abera, C. Viollet +4 more
2017Scientific Reports1 citations Open access
Semantic Scholar
Socio-cultural adaptation and standardization of Dubois' five words testing in a population of normal subject in Mali, West Africa
C. Guinto, T. Coulibaly, Z. Koné, S. Coulibaly +10 more
2016eNeurologicalSci1 citations Open access
Semantic Scholar
Short ArticleMicroRNA Pathways Modulate Polyglutamine-Induced Neurodegeneration
Julide Bilen, Nan Liu, Barrington G. Burnett, R. Pittman +1 more
20061 citations
Semantic Scholar
SMN deficiency causes cardiac adrenergic insensitivity via activation of the cAMP-PKA pathway.
Cecelia C. Mangione, Michael G Klein, N. Fatima, Natalya Belous +5 more
2026Neurobiology of Disease0 citations Open access
Semantic Scholar
The survival motor neuron protein: structure, functions, stability, and therapeutic targeting
Bradley R. Smith, Rachel Massalee, Mason Mayer, Brendan M. Melvin +3 more
2026Frontiers in Cellular Neuroscience0 citations Open access
Semantic Scholar

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