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Visar resultat 1 - 5 av 28 avhandlingar som matchar ovanstående sökkriterier.
1. SOD1´s Law : An Investigation of ALS Provoking Properties in SOD1
Sammanfattning : Proteins are the most important molecules in the cell since they take care of most of the biological functions which resemble life. To ensure that everything is working properly the cell has a rigorous control system to monitor the proper function of its proteins and sends old or dysfunctional proteins for degradation. LÄS MER
2. SOD1 misfolding and aggregation in ALS : in the light of conformation-specific antibodies
Sammanfattning : Mutations in the superoxide dismutase 1 (SOD1) gene are linked to the progressive neurodegenerative disease amyotrophic lateral sclerosis (ALS). ALS-associated mutations affect the stability of the SOD1 protein and promote its unfolding. As a consequence, disordered SOD1 species can misfold and accumulate into insoluble aggregates. LÄS MER
3. SOD1 Aggregation : Relevance of thermodynamic stability
Sammanfattning : Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the upper and lower motor neurons causing muscle atrophy and paralysis followed by death. Aggregates containing superoxide dismutase (SOD1) are found as pathological hallmark in diseased ALS patients. LÄS MER
4. Structural investigation of SOD1 aggregates in ALS : identification of prion strains using anti-peptide antibodies
Sammanfattning : Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative syndrome characterized by progressive degeneration of motor neurons that result in muscle wasting. The symptoms advance gradually to paralysis and eventually death. Most patients suffer from sporadic ALS (sALS) but 10% report a familial predisposition. LÄS MER
5. Using patient-derived cell models to investigate the role of misfolded SOD1 in ALS
Sammanfattning : Protein misfolding and aggregation underlie several neurodegenerative proteinopathies including amyotrophic lateral sclerosis (ALS). Superoxide dismutase 1 (SOD1) was the first gene found to be associated with familial ALS. LÄS MER