Sökning: "disulfide-reduced"

Hittade 4 avhandlingar innehållade ordet disulfide-reduced.

1. 1. Superoxide dismutase 1 and amyotrophic lateral sclerosis

   Författare :P. Andreas Jonsson; Stefan L. Marklund; David R. Borchelt; Umeå universitet; []
   Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; Neurosciences; aggregates; ALS; amyotrophic lateral sclerosis; cerebrospinal fluid; disulfide-reduced; inclusions; misfolded; protective factor; SOD1; transgenic; Neurovetenskap; Neurology; Neurologi; Clinical Chemistry; klinisk kemi;

   Sammanfattning : Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons in the spinal cord, brain stem and motor cortex, leading to paralysis, respiratory failure and death. In about 5% of ALS cases, the disease is associated with mutations in the CuZn-superoxide dismutase (hSOD1) gene. LÄS MER

3. 2. Misfolded superoxide dismutase-1 in amyotrophic lateral sclerosis

   Författare :Per Zetterström; Stefan L Marklund; Lawrence J. Hayward; Umeå universitet; []
   Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; SOD1; protein misfolding; SOD1 conformation; disulfide-reduced; transgenic mice; cerebrospinal fluid; protein-protein interaction; antibodies; Clinical chemistry; Klinisk kemi; Clinical Chemistry; klinisk kemi;

   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

4. 3. Proteins in Vacuo : Molecular dynamics studies of hen egg white lysozyme submitted to unfolding and relaxation conditions

   Författare :Ignasi Velázquez i Castelló; Uppsala universitet; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; Physics; Fysik; Physics; Fysik; Physical Chemistry; fysikalisk kemi;

   Sammanfattning : Electrically neutral and charged lysozyme molecules were studied with molecular dynamics (MD) techniques. Denaturation and renaturation conditions were simulated for the system in vacuo. Experimental observations of biomolecules in vacuo were related to the conformational space accessible under unfolding-relaxation conditions. LÄS MER

5. 4. 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