Sökning: "Misfolded species"

Visar resultat 1 - 5 av 15 avhandlingar innehållade orden Misfolded species.

1. 1. SOD, ORF and ALS: On the role of SOD1 and C9ORF72 in the pathogenesis of ALS

   Författare :Isil Keskin; Peter M. Andersen; Lars Lannfelt; Umeå universitet; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; MEDICAL AND HEALTH SCIENCES; ALS; SOD1; Misfolded species;

   Sammanfattning : Amyotrophic lateral sclerosis (ALS) is characterized by adult-onset degeneration of upper and lower motor neurons. Symptoms begin focally in one muscle and then spread contiguously, resulting in progressive paralysis and death from respiratory failure. LÄS MER

3. 2. 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

4. 3. The Alzheimer Aβ Peptide : Identification of Properties Distinctive for Toxic Prefibrillar Species

   Författare :Anna-Lena Göransson; Jenny Presto; Linköpings universitet; []
   Nyckelord :;

   Sammanfattning : Proteins must have specific conformations to function correctly inside cells. However, sometimes they adopt the wrong conformation, causing dysfunction and disease. A number of amyloid diseases are caused by misfolded proteins that form amyloid fibrils. One such disease is Alzheimer’s disease (AD). 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

7. 5. Understanding the dual nature of lysozyme: part villain – part hero : A Drosophila melanogaster model of lysozyme amyloidosis

   Författare :Linda Helmfors; Ann-Christin Brorsson; Bengt-Harald (Nalle) Jonsson; R. Luke Wiseman; Linköpings universitet; []
   Nyckelord :;

   Sammanfattning : Amyloid proteins are a distinct class of proteins that can misfold into β-sheet rich structures that later mature to form the characteristic species known as amyloid fibrils, and accumulate in tissues in the human body. The misfolding event is often caused by mutations (or outer factors such as changes in pH) that destabilize the native protein structure. LÄS MER