Mechanisms for neuronal degeneration in amyotrophic lateral sclerosis and in models of motor neuron death (Review).

2000 ◽  
Author(s):  
L J Martin ◽  
A C Price ◽  
A Kaiser ◽  
A Y Shaikh ◽  
Z Liu
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Neuronal Degeneration ◽  
Neuron Death ◽  
Motor Neuron Death ◽  
Lateral Sclerosis

scholarly journals Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death

2015 ◽  
Vol 130 (1) ◽  
pp. 49-61 ◽  
Author(s):  
Shangxi Xiao ◽  
Teresa Sanelli ◽  
Helen Chiang ◽  
Yulong Sun ◽  
Avijit Chakrabartty ◽  
...  
Keyword(s):  
Molecular Weight ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Low Molecular Weight ◽  
Neuron Death ◽  
Motor Neuron Death ◽  
Lateral Sclerosis

Ferroptosis mediates selective motor neuron death in amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Taide Wang ◽  
Doris Tomas ◽  
Nirma D. Perera ◽  
Brittany Cuic ◽  
Sophia Luikinga ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Neuron Death ◽  
Motor Neuron Death ◽  
Lateral Sclerosis

scholarly journals Extracellular ATP and the P2X7 receptor in astrocyte-mediated motor neuron death: implications for amyotrophic lateral sclerosis

2010 ◽  
Vol 7 (1) ◽  
pp. 33 ◽  
Author(s):  
Mandi Gandelman ◽  
Hugo Peluffo ◽  
Joseph S Beckman ◽  
Patricia Cassina ◽  
Luis Barbeito
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
P2x7 Receptor ◽  
Extracellular Atp ◽  
Neuron Death ◽  
Motor Neuron Death ◽  
Lateral Sclerosis

scholarly journals Fighting Amyotrophic Lateral Sclerosis: In-Silico Molecular Docking Study of SFPQ Protein to Identify a Potential Therapeutic Compound

2021 ◽  
Author(s):  
Omkar Kovvali
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
In Silico ◽  
Rna Binding ◽  
Nuclear Dna ◽  
Death Sentence ◽  
Neuron Death ◽  
Molecular Docking Study ◽  
Motor Neuron Death ◽  
Lateral Sclerosis

Abstract Amyotrophic Lateral Sclerosis, known as ALS, is a neurodegenerative disease that passes the death sentence on 15 new people every day, leaving a patient struggling to move their fingers. At the molecular level, motor neuron death causes the inability for the muscles to function as normal. After researchers studied motor neuron death in ALS patients’ cells, they found that in all diseased cells, the SFPQ protein, a vital nuclear DNA/RNA binding protein, left the nucleus (its original location) and ended up in the cytoplasm. Since this defect was a molecular hallmark of ALS, solving this problem could stop motor neuron death. To treat this molecular hallmark, the in-silico process was used to sift through thousands of potential molecules to find the best one to use. The in-silico method was used to identify a lead molecule (NLS 551) that passed all the in-silico tests and brought the SFPQ protein back into the nucleus. If successful, this identified molecule could serve as the base for an ALS treatment.

Amyotrophic Lateral Sclerosis

2017 ◽  
Author(s):  
Laura Ferraiuolo ◽  
Stephen J. Kolb
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Molecular Basis ◽  
Biological Characteristics ◽  
Molecular Pathways ◽  
Neuron Death ◽  
Neuron Loss ◽  
Motor Neuron Death ◽  
Motor Neuron Loss ◽  
Lateral Sclerosis

An overriding mystery of ALS pathogenesis orbits around the molecular basis of selective motor neuron vulnerability and clouds our view. There are likely mechanisms involved in the initiation of motor neuron loss and mechanisms involved in the progression of motor neuron loss once initiated. Motor neuron vulnerability is likely related to the unique biological characteristics of these cells. This chapter introduces central molecular pathways that appear to be involved in the pathogenesis of ALS, and highlights why dysregulation of these mechanisms could lead to motor neuron death. Indeed, there are likely mechanisms involved in the initiation of motor neuron loss and mechanisms involved in the progression of motor neuron loss once initiated. Our task is to determine those mechanisms that are relevant to ALS pathogenesis that may be targeted therapeutically to prevent onset and/or halt progression.

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