Selective loss of alpha motor neurons with sparing of gamma motor neurons and spinal cord cholinergic neurons in a mouse model of spinal muscular atrophy
RA Powis, TH Gillingwater - Journal of anatomy, 2016 - Wiley Online Library
Spinal muscular atrophy (SMA) is a neuromuscular disease characterised primarily by loss
of lower motor neurons from the ventral grey horn of the spinal cord and proximal muscle …
of lower motor neurons from the ventral grey horn of the spinal cord and proximal muscle …
Morphological characteristics of motor neurons do not determine their relative susceptibility to degeneration in a mouse model of severe spinal muscular atrophy
SR Thomson, JE Nahon, CA Mutsaers, D Thomson… - PloS one, 2012 - journals.plos.org
Spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality, resulting
primarily from the degeneration and loss of lower motor neurons. Studies using mouse …
primarily from the degeneration and loss of lower motor neurons. Studies using mouse …
SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy
G Hunter, A Aghamaleky Sarvestany… - Human molecular …, 2014 - academic.oup.com
Low levels of survival of motor neuron (SMN) protein lead to spinal muscular atrophy (SMA).
The major pathological hallmark of SMA is a loss of lower motor neurons from spinal cord …
The major pathological hallmark of SMA is a loss of lower motor neurons from spinal cord …
Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction
RG Gogliotti, KA Quinlan, CB Barlow… - Journal of …, 2012 - Soc Neuroscience
The loss of motor neurons (MNs) is a hallmark of the neuromuscular disease spinal
muscular atrophy (SMA); however, it is unclear whether this phenotype autonomously …
muscular atrophy (SMA); however, it is unclear whether this phenotype autonomously …
Motor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse models
Y Hua, YH Liu, K Sahashi, F Rigo… - Genes & …, 2015 - genesdev.cshlp.org
Survival of motor neuron (SMN) deficiency causes spinal muscular atrophy (SMA), but the
pathogenesis mechanisms remain elusive. Restoring SMN in motor neurons only partially …
pathogenesis mechanisms remain elusive. Restoring SMN in motor neurons only partially …
Functional abnormalities of cerebellum and motor cortex in spinal muscular atrophy mice
A Tharaneetharan, M Cole, B Norman, NC Romero… - Neuroscience, 2021 - Elsevier
Spinal muscular atrophy (SMA) is a devastating genetic neuromuscular disease. Diffuse
neuropathology has been reported in SMA patients and mouse models, however, functional …
neuropathology has been reported in SMA patients and mouse models, however, functional …
Spinal astrocyte dysfunction drives motor neuron loss in late-onset spinal muscular atrophy
LI Schmitt, C David, R Steffen, S Hezel, A Roos… - Acta …, 2023 - Springer
Spinal muscular atrophy (SMA) is a progressive neuromuscular disorder caused by a loss of
the survival of motor neuron 1 (SMN1) gene, resulting in a loss of spinal motor neurons …
the survival of motor neuron 1 (SMN1) gene, resulting in a loss of spinal motor neurons …
Mechanisms involved in spinal cord central synapse loss in a mouse model of spinal muscular atrophy
O Tarabal, V Caraballo-Miralles… - … of Neuropathology & …, 2014 - academic.oup.com
Motoneuron (MN) cell death is the histopathologic hallmark of spinal muscular atrophy
(SMA), although MN loss seems to be a late event. Conversely, disruption of afferent …
(SMA), although MN loss seems to be a late event. Conversely, disruption of afferent …
Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in …
X Paez-Colasante, B Seaberg, TL Martinez, L Kong… - PloS one, 2013 - journals.plos.org
In the inherited childhood neuromuscular disease spinal muscular atrophy (SMA), lower
motor neuron death and severe muscle weakness result from the reduction of the …
motor neuron death and severe muscle weakness result from the reduction of the …
Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy
The childhood motor neuron disease spinal muscular atrophy (SMA) results from reduced
expression of the survival motor neuron (SMN) gene. Previous studies using in vitro model …
expression of the survival motor neuron (SMN) gene. Previous studies using in vitro model …