Diverse role of survival motor neuron protein

RN Singh, MD Howell, EW Ottesen… - Biochimica et Biophysica …, 2017 - Elsevier
Abstract The multifunctional Survival Motor Neuron (SMN) protein is required for the survival
of all organisms of the animal kingdom. SMN impacts various aspects of RNA metabolism …

Spinal muscular atrophy

A D'Amico, E Mercuri, FD Tiziano, E Bertini - Orphanet journal of rare …, 2011 - Springer
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease
characterized by degeneration of alpha motor neurons in the spinal cord, resulting in …

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 …

[HTML][HTML] Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ …

RS Finkel, E Mercuri, OH Meyer, AK Simonds… - Neuromuscular …, 2018 - Elsevier
This is the second half of a two-part document updating the standard of care
recommendations for spinal muscular atrophy published in 2007. This part includes updated …

Biodistribution of onasemnogene abeparvovec DNA, mRNA and SMN protein in human tissue

G Thomsen, AHM Burghes, C Hsieh, J Do, BTT Chu… - Nature medicine, 2021 - nature.com
Spinal muscular atrophy type 1 (SMA1) is a debilitating neurodegenerative disease resulting
from survival motor neuron 1 gene (SMN1) deletion/mutation. Onasemnogene abeparvovec …

Peripheral SMN restoration is essential for long-term rescue of a severe spinal muscular atrophy mouse model

Y Hua, K Sahashi, F Rigo, G Hung, G Horev… - Nature, 2011 - nature.com
Spinal muscular atrophy (SMA) is a motor neuron disease and the leading genetic cause of
infant mortality; it results from loss-of-function mutations in the survival motor neuron 1 …

Emerging therapies and challenges in spinal muscular atrophy

MA Farrar, SB Park, S Vucic, KA Carey… - Annals of …, 2017 - Wiley Online Library
Spinal muscular atrophy (SMA) is a hereditary neurodegenerative disease with severity
ranging from progressive infantile paralysis and premature death (type I) to limited motor …

Childhood spinal muscular atrophy: controversies and challenges

E Mercuri, E Bertini, ST Iannaccone - The Lancet Neurology, 2012 - thelancet.com
Spinal muscular atrophy is an autosomal recessive disorder characterised by degeneration
of motor neurons in the spinal cord and is caused by mutations of the survival of motor …

Spinal muscular atrophy: going beyond the motor neuron

G Hamilton, TH Gillingwater - Trends in molecular medicine, 2013 - cell.com
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by abnormally low
cellular levels of the ubiquitous protein SMN. Traditionally, reduced levels of SMN were …

Spinal muscular atrophies

BT Darras, JA Markowitz, UR Monani… - … disorders of infancy …, 2015 - Elsevier
Spinal muscular atrophies (SMAs) are hereditary degenerative disorders of lower motor
neurons associated with progressive muscle weakness and atrophy. Proximal 5q SMA is …