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 …

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 …

[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 …

At the “junction” of spinal muscular atrophy pathogenesis: the role of neuromuscular junction dysfunction in SMA disease progression

BB Goulet, R Kothary, RJ Parks - Current molecular medicine, 2013 - ingentaconnect.com
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 …

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 …

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 …

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 …

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 …

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) …

[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 …