Spinal muscular atrophy: mutations, testing, and clinical relevance

MC Keinath, DE Prior, TW Prior - The Application of Clinical …, 2021 - Taylor & Francis
Spinal muscular atrophy (SMA) is a heritable neuromuscular disorder that causes
degeneration of the alpha motor neurons from anterior horn cells in the spinal cord, which …

[HTML][HTML] Genomic Variability in the Survival Motor Neuron Genes (SMN1 and SMN2): Implications for Spinal Muscular Atrophy Phenotype and Therapeutics …

MER Butchbach - International Journal of Molecular Sciences, 2021 - mdpi.com
Spinal muscular atrophy (SMA) is a leading genetic cause of infant death worldwide that is
characterized by loss of spinal motor neurons leading to muscle weakness and atrophy …

Base editing rescue of spinal muscular atrophy in cells and in mice

M Arbab, Z Matuszek, KM Kray, A Du, GA Newby… - Science, 2023 - science.org
Spinal muscular atrophy (SMA), the leading genetic cause of infant mortality, arises from
survival motor neuron (SMN) protein insufficiency resulting from SMN1 loss. Approved …

[PDF][PDF] Comprehensive SMN1 and SMN2 profiling for spinal muscular atrophy analysis using long-read PacBio HiFi sequencing

X Chen, J Harting, E Farrow, I Thiffault… - The American Journal of …, 2023 - cell.com
Spinal muscular atrophy, a leading cause of early infant death, is caused by bi-allelic
mutations of SMN1. Sequence analysis of SMN1 is challenging due to high sequence …

[HTML][HTML] Spinal muscular atrophy diagnosis and carrier screening from genome sequencing data

X Chen, A Sanchis-Juan, CE French, AJ Connell… - Genetics in …, 2020 - nature.com
Purpose Spinal muscular atrophy (SMA), caused by loss of the SMN1 gene, is a leading
cause of early childhood death. Due to the near identical sequences of SMN1 and SMN2 …

[HTML][HTML] Onasemnogene abeparvovec gene replacement therapy for the treatment of spinal muscular atrophy: a real-world observational study

I Bitetti, V Lanzara, G Margiotta, A Varone - Gene Therapy, 2023 - nature.com
Spinal muscular atrophy (SMA) is a genetically inherited recessive neuromuscular disease
that causes muscular atrophy and weakness. Onasemnogene abeparvovec (formerly AVXS …

[HTML][HTML] Comprehensive analysis of spinal muscular atrophy: SMN1 copy number, intragenic mutation, and 2+ 0 carrier analysis by third-generation sequencing

S Li, X Han, Y Xu, C Chang, L Gao, J Li, Y Lu… - The Journal of Molecular …, 2022 - Elsevier
Population-wide carrier screening for spinal muscular atrophy (SMA) is recommended by
the American College of Medical Genetics and Genomics. However, the methods used …

[HTML][HTML] Biomarkers and the development of a personalized medicine approach in spinal muscular atrophy

DST Kariyawasam, A D'Silva, C Lin, MM Ryan… - Frontiers in …, 2019 - frontiersin.org
Recent unprecedented advances in treatment for spinal muscular atrophy (SMA) enabled
patients to access the first approved disease modifying therapy for the condition. There are …

244th ENMC international workshop: newborn screening in spinal muscular atrophy May 10–12, 2019, Hoofdorp, The Netherlands

T Dangouloff, A Burghes, EF Tizzano, L Servais… - Neuromuscular …, 2020 - Elsevier
A total of 19 participants including clinicians, newborn screening specialists, economists,
geneticists, patients and patient advocates, and industry representatives from 12 countries …

Practical guidelines to manage discordant situations of SMN2 copy number in patients with spinal muscular atrophy

I Cuscó, S Bernal, L Blasco-Pérez, M Calucho… - Neurology …, 2020 - AAN Enterprises
Objective Assessment of SMN2 copy number in patients with spinal muscular atrophy (SMA)
is essential to establish careful genotype-phenotype correlations and predict disease …