Sökning: "misfolded protein"

Visar resultat 31 - 35 av 73 avhandlingar innehållade orden misfolded protein.

1. 31. The role of the ubiquitin-proteasome system in neurodegenerative disorders

   Författare :Lisette Gerridina Gezina Catharina Verhoef; Karolinska Institutet; Karolinska Institutet; []
   Nyckelord :Degradation; green fluorescent protein GFP ; neurodegeneration; polyglutamine; proteasome; UBB 1; ubiquitin;

   Sammanfattning : Neurodegenerative disorders are a heterogeneous group of clinically and pathologically diverse diseases. The diseases are characterised by selective loss of neurons. in specific regions of the brain. The result is disruption of motor, sensory or cognitive systems, leading to severe disability of the patients. LÄS MER

3. 32. Characterization of p58 and rab1p, two proteins operating at the interface of the endoplasmic reticulum and the Golgi complex

   Författare :Ulla Lahtinen; Karolinska Institutet; Karolinska Institutet; []
   Nyckelord :protein transport; rab1p; p58; animal lectin; intermediate compartment; endoplasmic reticulum; Golgi complex;

   Sammanfattning : Endomembranes participating in the early stages in exocytosis consist of the endoplasmic reticulum (ER), the Golgi complex, and vesicular transport intermediates at their interface. These vesicular transport intermediates were first visualized by following the synchronized transport of a viral membrane protein from the ER to the Golgi complex, which occurs at slower rates at reduced temperatures causing an accumulation of transport vesicles. LÄS MER

4. 33. Functional studies of the ubiquitin proteasome system using GFP based reporters

   Författare :Kristina Lindsten; Karolinska Institutet; Karolinska Institutet; []
   Nyckelord :Ubiquitin; proteasome; green fluorescent protein GFP ; degradation; inhibitor; Gly-Ala repeat; neurodegencration; poly-Gln aggregates.;

   Sammanfattning : Ubiquitin-dependent proteasomal degradation is of paramount importance for cellular processes such as cell cycle progression, transcriptional regulation, apoptosis and disposal of misfolded and aberrant proteins. Moreover the ubiquitin-proteasome system is the main producer of peptides used by major histocompatibility complex class 1 for antigen presentation. LÄS MER

5. 34. Folding and retention of rotavirus glycoproteins in the endoplasmic reticulum

   Författare :Ali Mirazimi; Karolinska Institutet; Karolinska Institutet; []
   Nyckelord :ER; rotavirus; VP7; NSP4; oligosaccharide processing; N-linked glycosylation; protein disulfide isomerase; calnexin; ATP; ER retention;

   Sammanfattning : The endoplasmic reticulum (ER) is a membrane bound organelle composed of a network of cisternae stretching throughout the cytoplasm. Functionally, the ER performs many essential processes, such as glycosylation, and biosynthesis of lipids; it provides a redox potential condition for forming S-S linkages, and a quality control system, and functions as an intracellular calcium store. LÄS MER

7. 35. 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