Sökning: "Thomas Brännström"
Visar resultat 6 - 10 av 10 avhandlingar innehållade orden Thomas Brännström.
6. Misfolded superoxide dismutase-1 in sporadic and familial Amyotrophic Lateral Sclerosis
Sammanfattning : Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative syndrome of unknown etiology that most commonly affects people in middle and high age. The hallmark of ALS is a progressive and simultaneous loss of upper and lower motor neurons in the central nervous system that leads to a progressive muscle atrophy, paralysis and death usually by respiratory failure. LÄS MER
7. 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
8. 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
9. 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
10. Extraocular Muscles in Amyotrophic Lateral Sclerosis
Sammanfattning : Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease of motor neurons characterized by muscle paralysis and death within 3-5 years of onset. However, due to unknown mechanisms, the extraocular muscles (EOMs) remain remarkably unaffected. LÄS MER