Sökning: "SOD1"

Visar resultat 1 - 5 av 28 avhandlingar innehållade ordet SOD1.

1. 1. SOD1´s Law : An Investigation of ALS Provoking Properties in SOD1

   Författare :Roberth Byström; Gerhard Gröbner; Stefan Marklund; Mikael Oliveberg; Bengt-Harald Jonsson; Umeå universitet; []
   Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; amyotrophic lateral sclerosis; SOD1; protein folding; membrane interaction; aggregates; survival time; repulsive charge; Biochemistry; Biokemi;

   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

3. 2. SOD1 misfolding and aggregation in ALS : in the light of conformation-specific antibodies

   Författare :Manuela Lehmann; Jonathan D. Gilthorpe; Stefan L. Marklund; Thomas Brännström; Ulrika Nordström; Elizabeth Fisher; Umeå universitet; []
   Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; antibodies; SOD1; disordered SOD1; patient-derived models; low oxygen tension; Immunotherapy;

   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

4. 3. SOD1 Aggregation : Relevance of thermodynamic stability

   Författare :Lisa Lang; Mikael Oliveberg; Michele Vendruscolo; Stockholms universitet; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; Aggregation; misfolding; Superoxide dismutase SOD1 ; Amyotrophic lateral sclerosis ALS ; unfolding; thermodynamic stability; Biochemistry; biokemi;

   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

5. 4. Structural investigation of SOD1 aggregates in ALS : identification of prion strains using anti-peptide antibodies

   Författare :Johan Bergh; Thomas Brännström; Stefan L. Marklund; Peter M. Andersen; Joakim Bergström; Umeå universitet; []
   Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; SOD1; prion; motor neuron disease; neurodegeneration; strain; seeding; protein aggregation; transgenic mice; peptide antibodies; Neurology; neurologi;

   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

7. 5. Using patient-derived cell models to investigate the role of misfolded SOD1 in ALS

   Författare :Elin Forsgren; Jonathan Gilthorpe; Stefan Marklund; Peter Andersen; Thomas Brännström; Ulrika Nordström; Séverine Boillée; Umeå universitet; []
   Nyckelord :ALS; SOD1; patient-derived models; induced pluripotent stem cells; motor neurons; astrocytes; 20S proteasome low oxygen tension; misfolded SOD1; Neurology; neurologi;

   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