Sökning: "Prion"

Visar resultat 1 - 5 av 51 avhandlingar innehållade ordet Prion.

  1. 1. 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 :MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; 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

  2. 2. Prion-infection and Cellular Signaling : Influence of scrapie-infection on lipid raft-associated proteins

    Författare :Hanna Gyllberg; Katarina Bedecs; Albert Taraboulos; Stockholms universitet; []
    Nyckelord :NATURAL SCIENCES; NATURVETENSKAP; NATURVETENSKAP; NATURAL SCIENCES; Biochemistry; Biokemi; biokemi; Biochemistry;

    Sammanfattning : Prion diseases are a group of fatal neurodegenerative diseases affecting almost all mammals including humans. The diseases are caused by formation of the misfolded isoform of the cellular prion protein (PrPC) to the disease causing PrPSc. LÄS MER

  3. 3. Novel endogenous mechanisms of complement regulation - A delicate balance

    Författare :Andreas Holmér; Malmö Klinisk kemi; []
    Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; short leucine rich glycoprotein; factor H; C1q; age-related macular degeneration; C4b-binding protein; prion disease; prion protein; Complement regulation; C-reactive protein;

    Sammanfattning : In this thesis, the biochemical mechanisms for complement activation by endogenous proteins are explored. The short leucine-rich repeat proteins (SLRPs) help organise extracellular matrices. We found that several SLRPs bind C1q and of these, fibromodulin and osteoadherin trigger complement. LÄS MER

  4. 4. SOD1 prions transmit templated aggregation and fatal ALS-like disease

    Författare :Elaheh Ekhtiari Bidhendi; Thomas Brännström; Stefan L. Marklund; Peter M. Andersen; Inger Nennesmo; Umeå universitet; []
    Nyckelord :MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICAL AND HEALTH SCIENCES; ALS; amyotrophic lateral sclerosis; SOD1; prion; neurodegeneration; strain; seeding; protein misfolding; protein aggregation; propagation; transgenic mice; Pathology; patologi; Neurology; neurologi;

    Sammanfattning : Amyotrophic lateral sclerosis (ALS) is an adult-onset fatal neurodegenerative disease characterized by a progressive degeneration of the upper and lower motor neurons. The resulting paresis begins focally, usually in one muscle, and spreads contiguously, leading to muscle wasting, progressive paralysis and eventually death. LÄS MER

  5. 5. Protein folding variants, for better or for worse? The lesson learned from HAMLET

    Författare :Malin Svensson; immunologi och glykobiologi - MIG Avdelningen för mikrobiologi; []
    Nyckelord :MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; Clinical biology; prion; apo; fatty acid; apoptosis; lactalbumin; folding variant; Klinisk biologi;

    Sammanfattning : By varying its secondary or tertiary structure a single protein can attain new biological functions. The amyloid forming proteins are the best known examples where a change in conformation leads to the formation of amyloid fibrils that accumulate in tissues and cause disease. LÄS MER