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Samantha Powers

Explore the profile of Samantha Powers including associated specialties, affiliations and a list of published articles. Areas
Snapshot
Articles 6
Citations 53
Followers 0
Related Specialties
Biology
Cell Biology
Molecular Biology
Top 10 Co-Authors
Shibi Likhite
Xiaojin Zhang
Kathrin C Meyer
Kathrin Meyer
Cassandra Dennys
Nicolas Wein
Patricia Cogram
Meysam Ganjibakhsh
Mohini Parvate
Roger B Sher
Published In
Toxicol Sci
Biology (Basel)
Aging Cell
Mol Ther
Cell Rep
Affiliations
Soon will be listed here.
Recent Articles
1.
MeCP2 gene therapy ameliorates disease phenotype in mouse model for Pitt Hopkins syndrome
Dennys C, Vermudez S, Deacon R, Sierra-Delgado J, Rich K, Zhang X, et al.
Neurotherapeutics . 2024 Jun; 21(5):e00376. PMID: 38876822
The neurodevelopmental disorder Pitt Hopkins syndrome (PTHS) causes clinical symptoms similar to Rett syndrome (RTT) patients. However, RTT is caused by MECP2 mutations whereas mutations in the TCF4 gene lead...
2.
Novel MECP2 gene therapy is effective in a multicenter study using two mouse models of Rett syndrome and is safe in non-human primates
Powers S, Likhite S, Gadalla K, Miranda C, Huffenberger A, Dennys C, et al.
Mol Ther . 2023 Jul; 31(9):2767-2782. PMID: 37481701
The AAV9 gene therapy vector presented in this study is safe in mice and non-human primates and highly efficacious without causing overexpression toxicity, a major challenge for clinical translation of...
3.
In Vitro Modeling as a Tool for Testing Therapeutics for Spinal Muscular Atrophy and IGHMBP2-Related Disorders
Sierra-Delgado J, Sinha-Ray S, Kaleem A, Ganjibakhsh M, Parvate M, Powers S, et al.
Biology (Basel) . 2023 Jun; 12(6). PMID: 37372153
Spinal Muscular Atrophy (SMA) is the leading genetic cause of infant mortality. The most common form of SMA is caused by mutations in the SMN1 gene, located on 5q (SMA)....
4.
Mechanisms of IRF2BPL-related disorders and identification of a potential therapeutic strategy
Sinha Ray S, Dutta D, Dennys C, Powers S, Roussel F, Lisowski P, et al.
Cell Rep . 2022 Dec; 41(10):111751. PMID: 36476864
The recently discovered neurological disorder NEDAMSS is caused by heterozygous truncations in the transcriptional regulator IRF2BPL. Here, we reprogram patient skin fibroblasts to astrocytes and neurons to study mechanisms of...
5.
Directly converted astrocytes retain the ageing features of the donor fibroblasts and elucidate the astrocytic contribution to human CNS health and disease
Gatto N, Souza C, Shaw A, Bell S, Myszczynska M, Powers S, et al.
Aging Cell . 2020 Dec; 20(1):e13281. PMID: 33314575
Astrocytes are highly specialised cells, responsible for CNS homeostasis and neuronal activity. Lack of human in vitro systems able to recapitulate the functional changes affecting astrocytes during ageing represents a...
6.
Editor's Highlight: Embryonic Exposure to the Environmental Neurotoxin BMAA Negatively Impacts Early Neuronal Development and Progression of Neurodegeneration in the Sod1-G93R Zebrafish Model of Amyotrophic Lateral Sclerosis
Powers S, Kwok S, Lovejoy E, Lavin T, Sher R
Toxicol Sci . 2017 Jan; 157(1):129-140. PMID: 28123103
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder leading to progressive paralysis and death within 2-5 years after diagnosis. Sporadic cases (SALS) comprise approximately 90% of cases with the remaining...