Plastin 3 rescues cell surface translocation and activation of TrkB in spinal muscular atrophy
L Hennlein, H Ghanawi, F Gerstner… - Journal of Cell …, 2023 - rupress.org
Plastin 3 (PLS3) is an F-actin-bundling protein that has gained attention as a modifier of
spinal muscular atrophy (SMA) pathology. SMA is a lethal pediatric neuromuscular disease …
spinal muscular atrophy (SMA) pathology. SMA is a lethal pediatric neuromuscular disease …
Molecular factors involved in spinal muscular atrophy pathways as possible disease-modifying candidates
MA Maretina, GY Zheleznyakova, KM Lanko… - Current …, 2018 - ingentaconnect.com
Spinal Muscular Atrophy (SMA) is a neuromuscular disorder caused by mutations in the
SMN1 gene. Being a monogenic disease, it is characterized by high clinical heterogeneity …
SMN1 gene. Being a monogenic disease, it is characterized by high clinical heterogeneity …
[HTML][HTML] Bifunctional RNAs targeting the intronic splicing silencer N1 increase SMN levels and reduce disease severity in an animal model of spinal muscular atrophy
EY Osman, PF Yen, CL Lorson - Molecular Therapy, 2012 - cell.com
Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by loss of survival
motor neuron-1 (SMN1). A nearly identical copy gene, SMN2, is present in all SMA patients …
motor neuron-1 (SMN1). A nearly identical copy gene, SMN2, is present in all SMA patients …
At the “junction” of spinal muscular atrophy pathogenesis: the role of neuromuscular junction dysfunction in SMA disease progression
Spinal muscular atrophy (SMA) is caused by mutations that reduce the level of the survival
motor neuron protein (SMN) resulting in death of alpha-motor neurons, yet it is unclear why …
motor neuron protein (SMN) resulting in death of alpha-motor neurons, yet it is unclear why …
Beta-lactam antibiotic offers neuroprotection in a spinal muscular atrophy model by multiple mechanisms
M Nizzardo, M Nardini, D Ronchi, S Salani… - Experimental …, 2011 - Elsevier
Spinal muscular atrophy (SMA) is a devastating genetic motoneuron disease leading to
infant death. No effective therapy is currently available. It has been suggested that β-lactam …
infant death. No effective therapy is currently available. It has been suggested that β-lactam …
Calcium binding is essential for plastin 3 function in Smn-deficient motoneurons
AN Lyon, RH Pineda, T Hao… - Human molecular …, 2014 - academic.oup.com
The actin-binding and bundling protein, plastin 3 (PLS3), was identified as a protective
modifier of spinal muscular atrophy (SMA) in some patient populations and as a disease …
modifier of spinal muscular atrophy (SMA) in some patient populations and as a disease …
Glial Activation and Central Synapse Loss, but Not Motoneuron Degeneration, Are Prevented by the Sigma-1 Receptor Agonist PRE-084 in the Smn2B/− Mouse …
C Cerveró, A Blasco, O Tarabal… - … of Neuropathology & …, 2018 - academic.oup.com
Spinal muscular atrophy (SMA) is characterized by the loss of α-motoneurons (MNs) with
concomitant muscle denervation. MN excitability and vulnerability to disease are particularly …
concomitant muscle denervation. MN excitability and vulnerability to disease are particularly …
Laminin induced local axonal translation of β-actin mRNA is impaired in SMN-deficient motoneurons
R Rathod, S Havlicek, N Frank, R Blum… - Histochemistry and cell …, 2012 - Springer
Reduced levels of the SMN (survival of motoneuron) protein cause spinal muscular atrophy,
the main form of motoneuron disease in children and young adults. In cultured motoneurons …
the main form of motoneuron disease in children and young adults. In cultured motoneurons …
Temporal requirement for SMN in motoneuron development
LT Hao, PQ Duy, JD Jontes, M Wolman… - Human molecular …, 2013 - academic.oup.com
Proper function of the motor unit is dependent upon the correct development of dendrites
and axons. The infant/childhood onset motoneuron disease spinal muscular atrophy (SMA) …
and axons. The infant/childhood onset motoneuron disease spinal muscular atrophy (SMA) …
[HTML][HTML] BDNF/trkB Induction of Calcium Transients through Cav2.2 Calcium Channels in Motoneurons Corresponds to F-actin Assembly and Growth Cone Formation …
B Dombert, S Balk, P Lüningschrör… - Frontiers in molecular …, 2017 - frontiersin.org
Spontaneous Ca2+ transients and actin dynamics in primary motoneurons correspond to
cellular differentiation such as axon elongation and growth cone formation. Brain-derived …
cellular differentiation such as axon elongation and growth cone formation. Brain-derived …