Sökning: "misfolded"

Visar resultat 1 - 5 av 78 avhandlingar innehållade ordet misfolded.

  1. 1. Of mice and men : SOD1 associated human amyotrophic lateral sclerosis and transgenic mouse models

    Författare :Karin Sixtensdotter Graffmo; Thomas Brännström; Stefan Marklund; Elisabeth Englund; Umeå universitet; []
    Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; aggregates; ALS; amyotrophic lateral sclerosis; inclusions; misfolded; vacuolisation; SOD1; transgenic; Pathology; Patologi;

    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

  3. 2. 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

  4. 3. 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

  5. 4. 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

  7. 5. Induction of the heat-shock response by misfolded proteins

    Författare :Anna E. Masser; Claes Andréasson; Stockholms universitet; []
    Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; molekylär biovetenskap; Molecular Bioscience;

    Sammanfattning : Cells respond to stress by transcriptional changes intended for stress survival and adaption to further stresses. Heat shock is an intensively studied stress condition that induces in a plethora of physiological changes including increased protein misfolding. LÄS MER