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Physiopathology of ALS: from oxidative stress to RNA metabolism

Esposito, Sonia (2014) Physiopathology of ALS: from oxidative stress to RNA metabolism. Doctoral Thesis.

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Abstract

ALS is a progressive, degenerative disease characterized by selective death of motoneurons in brain and spinal cord. ALS is the most common motoneuron disease that in Sardinia affects 21 people per 100,000 inhabitants. Different ALS causative genes, including SOD1, FUS and TDP43 have been identified in both familial and sporadic ALS cases, suggesting a possible role of these proteins in both forms of the disease. Hypotheses on biology underlying both sporadic and familiar ALS outline a model in which non-competing mechanisms converge in various unsuccessful patterns to mediate motoneuron death. These progresses in understanding the pathological mechanisms in ALS have not been matched with an effective pharmacotherapy.
High−throughput experimental strategies (HTS) may provide a potent tool to identify new active drugs for ALS treatment able to interfere with the first cellular alterations, prior to the motoneuronal death. We tested several cellular models where specific markers of cell toxicity, induced by expression of ALS causative genes, may be used as a read-out for HT screening of compound libraries of different nature and we identified roGFP as a good candidate to monitor oxidative stress induced by SOD1.
Moreover we got some insight into the pathological mechanism of the Sardinian mutation of TDP43. This pathological mutant promotes cytoplasmic aggregates formations, which do not seem to be causally linked to cell death even if they are considered pathological hallmarks.

Item Type:Doctoral Thesis
ID Code:9491
Contributors:Crosio, Claudia
Publisher:Università degli studi di Sassari
Uncontrolled Keywords:Amyotrophic Lateral Sclerosis, SOD1, FUS, TDP43, neurodegeneration
Subjects:Area 05 - Scienze biologiche > BIO/11 Biologia molecolare
Divisions:001 Università di Sassari > 01-a Nuovi Dipartimenti dal 2012 > Scienze Biomediche
Cicli, scuole e corsi:Ciclo 26 > Scienze biomolecolari e Biotecnologiche > Biochimica e Biologia molecolare
Deposited On:01 Apr 2014 08:45

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