Sökning: "misfolded SOD1"
Visar resultat 1 - 5 av 8 avhandlingar innehållade orden misfolded SOD1.
1. 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
2. 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
3. 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
4. Of mice and men : SOD1 associated human amyotrophic lateral sclerosis and transgenic mouse models
Sammanfattning : Amyotrophic lateral sclerosis, ALS, is a progressive fatal neurodegenerative disorder affecting motor neurones in motor cortex, brain stem and spinal cord. This inevitably leads to paralysis, respiratory failure and death. LÄS MER
5. Misfolded superoxide dismutase-1 in amyotrophic lateral sclerosis
Sammanfattning : Amyotrophic lateral sclerosis (ALS) is a disease in which the motor neurons die in a progressive manner, leading to paralysis and muscle wasting. ALS is always fatal, usually through respiratory failure when the disease reaches muscles needed for breathing. Most cases are sporadic, but approximately 5–10% are familial. LÄS MER