Sökning: "disordered SOD1"
Hittade 5 avhandlingar innehållade orden disordered SOD1.
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
2. 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
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
4. Biophysical chemistry of the ALS-associated protein SOD1 : Implications for folding, aggregation and in-cell behaviour
Sammanfattning : Biophysical chemistry deals with the structural behavior, properties and molecular function of biological macromolecules. A long-standing challenge is here to establish how these macromolecular features change upon transfer from simplified conditions in vitro to the crowded and molecularly complex environment of live cells. LÄS MER
Sammanfattning : Although organisms have evolved sophisticated cellular mechanisms for regulating their various protein networks, proteins sometimes start to clump together in an uncontrolled way. The aggregation of proteins has been associated with a range of serious diseases. LÄS MER