scholarly journals Uncoupling of Protein Aggregation and Neurodegeneration in a Mouse Amyotrophic Lateral Sclerosis Model

2015 ◽  
Vol 15 (6) ◽  
pp. 339-349 ◽  
Author(s):  
Joo-Yong Lee ◽  
Yoshiharu Kawaguchi ◽  
Ming Li ◽  
Meghan Kapur ◽  
Su Jin Choi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Protein Aggregation ◽  
Lateral Sclerosis

scholarly journals Mitophagy Modulation, a New Player in the Race against ALS

2021 ◽  
Vol 22 (2) ◽  
pp. 740
Author(s):  
Enrique Madruga ◽  
Inés Maestro ◽  
Ana Martínez
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Protein Aggregation ◽  
Neurodegenerative Disease ◽  
Effective Treatment ◽  
Unknown Etiology ◽  
Drug Candidates ◽  
Relevant Evidence ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease that usually results in respiratory paralysis in an interval of 2 to 4 years. ALS shows a multifactorial pathogenesis with an unknown etiology, and currently lacks an effective treatment. The vast majority of patients exhibit protein aggregation and a dysfunctional mitochondrial accumulation in their motoneurons. As a result, autophagy and mitophagy modulators may be interesting drug candidates that mitigate key pathological hallmarks of the disease. This work reviews the most relevant evidence that correlate mitophagy defects and ALS, and discusses the possibility of considering mitophagy as an interesting target in the search for an effective treatment for ALS.

scholarly journals Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis

Brain ◽  
2009 ◽  
Vol 133 (1) ◽  
pp. 105-116 ◽  
Author(s):  
Adam K. Walker ◽  
Manal A. Farg ◽  
Chris R. Bye ◽  
Catriona A. McLean ◽  
Malcolm K. Horne ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Protein Aggregation ◽  
Protein Disulphide Isomerase ◽  
Lateral Sclerosis

scholarly journals Pyrimidine-2,4,6-trione Derivatives and Their Inhibition of Mutant SOD1-Dependent Protein Aggregation. Toward a Treatment for Amyotrophic Lateral Sclerosis

2011 ◽  
Vol 54 (7) ◽  
pp. 2409-2421 ◽  
Author(s):  
Guoyao Xia ◽  
Radhia Benmohamed ◽  
Jinho Kim ◽  
Anthony C. Arvanites ◽  
Richard I. Morimoto ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Protein Aggregation ◽  
Mutant Sod1 ◽  
Dependent Protein ◽  
Lateral Sclerosis
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