scholarly journals Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCFSlmb degron

2018 ◽  
Vol 29 (2) ◽  
pp. 96-110 ◽  
Author(s):  
Kelsey M. Gray ◽  
Kevin A. Kaifer ◽  
David Baillat ◽  
Ying Wen ◽  
Thomas R. Bonacci ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Protein Isoforms ◽  
Survival Motor Neuron Protein ◽  
Protein Levels ◽  
Motor Neuron Protein ◽  
Smn Protein ◽  
Oligomerization Domain

SMN protein levels inversely correlate with the severity of spinal muscular atrophy. The SCFSlmbE3 ligase complex interacts with a degron embedded within the C-terminal self-oligomerization domain of SMN. The findings elucidate a model whereby accessibility of the SMN degron is regulated by self-multimerization.

scholarly journals Whole blood survival motor neuron protein levels correlate with severity of denervation in spinal muscular atrophy

2020 ◽  
Vol 62 (3) ◽  
pp. 351-357 ◽  
Author(s):  
Christiano R. R. Alves ◽  
Ren Zhang ◽  
Alec J. Johnstone ◽  
Reid Garner ◽  
Eric J. Eichelberger ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Whole Blood ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Survival Motor Neuron Protein ◽  
Protein Levels ◽  
Motor Neuron Protein

scholarly journals Investigation of New Morpholino Oligomers to Increase Survival Motor Neuron Protein Levels in Spinal Muscular Atrophy

2018 ◽  
Vol 19 (1) ◽  
pp. 167 ◽  
Author(s):  
Agnese Ramirez ◽  
Sebastiano Crisafulli ◽  
Mafalda Rizzuti ◽  
Nereo Bresolin ◽  
Giacomo Comi ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Survival Motor Neuron Protein ◽  
Protein Levels ◽  
Motor Neuron Protein

scholarly journals Salbutamol inhibits ubiquitin-mediated survival motor neuron protein degradation in spinal muscular atrophy cells

2015 ◽  
Vol 4 ◽  
pp. 351-356 ◽  
Author(s):  
Nur Imma Fatimah Harahap ◽  
Dian Kesumapramudya Nurputra ◽  
Mawaddah Ar Rochmah ◽  
Ai Shima ◽  
Naoya Morisada ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Protein Degradation ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Survival Motor Neuron Protein ◽  
Motor Neuron Protein

Survival motor Neuron protein regulates apoptosis in anin vitro model of Spinal muscular atrophy

2008 ◽  
Vol 13 (1) ◽  
pp. 39-48 ◽  
Author(s):  
Graham C. Parker ◽  
Xingli Li ◽  
Roumen A. Anguelov ◽  
Gabor Toth ◽  
Adam Cristescu ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Survival Motor Neuron Protein ◽  
Motor Neuron Protein ◽  
Vitro Model

A novel evaluation method of survival motor neuron protein as a biomarker of spinal muscular atrophy

2015 ◽  
Vol 25 ◽  
pp. S193-S194
Author(s):  
M. Arakawa ◽  
R. Arakawa ◽  
R. Aoki ◽  
A. Nomoto ◽  
K. Saito ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Evaluation Method ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Survival Motor Neuron Protein ◽  
Motor Neuron Protein

scholarly journals HuD interacts with survival motor neuron protein and can rescue spinal muscular atrophy-like neuronal defects

2010 ◽  
Vol 20 (3) ◽  
pp. 553-579 ◽  
Author(s):  
Lisa Hubers ◽  
Hector Valderrama-Carvajal ◽  
Janik Laframboise ◽  
Janie Timbers ◽  
Gabriel Sanchez ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Muscular Atrophy ◽  
Survival Motor Neuron ◽  
Survival Motor Neuron Protein ◽  
Motor Neuron Protein
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