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

Histological Evidence of Protein Aggregation in Mutant SOD1 Transgenic Mice and in Amyotrophic Lateral Sclerosis Neural Tissues

2001 ◽  
Vol 8 (6) ◽  
pp. 933-941 ◽  
Author(s):  
Mitsunori Watanabe ◽  
Margaret Dykes-Hoberg ◽  
Valeria Cizewski Culotta ◽  
Donald L. Price ◽  
Philip C. Wong ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Transgenic Mice ◽  
Protein Aggregation ◽  
Histological Evidence ◽  
Mutant Sod1 ◽  
Neural Tissues ◽  
Lateral Sclerosis

Unraveling the Complexity of Amyotrophic Lateral Sclerosis: Recent Advances from the Transgenic Mutant SOD1 Mice

2010 ◽  
Vol 9 (4) ◽  
pp. 491-503 ◽  
Author(s):  
M. Peviani ◽  
I. Caron ◽  
C. Pizzasegola ◽  
F. Gensano ◽  
M. Tortarolo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mutant Sod1 ◽  
Recent Advances ◽  
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.

Differential effect of oxidative or excitotoxic stress on the transcriptional profile of amyotrophic lateral sclerosis-linked mutant SOD1 cultured neurons

2011 ◽  
Vol 89 (9) ◽  
pp. 1439-1450 ◽  
Author(s):  
Nadia Boutahar ◽  
Anne Wierinckx ◽  
Jean Philippe Camdessanche ◽  
Jean-Christophe Antoine ◽  
Evelyne Reynaud ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Differential Effect ◽  
Transcriptional Profile ◽  
Cultured Neurons ◽  
Mutant Sod1 ◽  
Lateral Sclerosis

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 Lack of TNF-alpha receptor type 2 protects motor neurons in a cellular model of amyotrophic lateral sclerosis and in mutant SOD1 mice but does not affect disease progression

2015 ◽  
Vol 135 (1) ◽  
pp. 109-124 ◽  
Author(s):  
Massimo Tortarolo ◽  
Antonio Vallarola ◽  
Dario Lidonnici ◽  
Elisa Battaglia ◽  
Francesco Gensano ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Motor Neurons ◽  
Receptor Type ◽  
Tnf Alpha ◽  
Cellular Model ◽  
Mutant Sod1 ◽  
Lateral Sclerosis
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