Bridging the gap: From protein misfolding to protein misfolding diseases
FEBS Letters, 2009 Protein misfolding and aggregation are pathognomic for a number of the most common age‐related degenerative diseases. Great progress has been made in studying protein aggregation in the test tube and also in replicating protein aggregation in vertebrate animal models of these diseases.
Luheshi, Leila M. +1 more
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HLA‑B27 misfolding and spondyloarthropathies [PDF]
Prion, 2009 HLA-B27 plays a central role in the pathogenesis of many spondyloarthropathies and in particular ankylosing spondylitis. The observation that the HLA-B27 heavy chain has a tendency to misfold has raised the possibility that associated diseases may belong in a rapidly expanding category of protein misfolding disorders. The synthesis of the HLA-B27 heavy
Robert A, Colbert +3 more
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iScience, 2020 Summary: The phenomenon of protein misfolding and aggregation is associated with a wide range of neurodegenerative conditions that cause progressive loss of function in specific regions of the human brain.
Rishika Kundra +2 more
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Cell Non-autonomous Proteostasis Regulation in Aging and Disease
Frontiers in Neuroscience, 2022 Aging is a risk factor for a number of diseases, being the more notorious ones perhaps neurodegenerative diseases such as Alzheimer’s and Parkinson’s. These and other age-related pathologies are often associated with accumulation of proteotoxic material ...
Joao Vasco Ferreira +2 more
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Prion degradation pathways: Potential for therapeutic intervention [PDF]
, 2015 Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
Goold, R, McKinnon, C, Tabrizi, SJ
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The common truncation variant in pancreatic lipase related protein 2 (PNLIPRP2) is expressed poorly and does not alter risk for chronic pancreatitis [PDF]
, 2018 A nonsense variant (p.W358X) of human pancreatic lipase related protein 2 (PNLIPRP2) is present in different ethnic populations with a high allele frequency.
Hegyi, Eszter +7 more
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Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging [PDF]
International Journal of Cell Biology, 2013 The misfolding, aggregation, and tissue accumulation of proteins are common events in diverse chronic diseases, known as protein misfolding disorders. Many of these diseases are associated with aging, but the mechanism for this connection is unknown. Recent evidence has shown that the formation and accumulation of protein aggregates may be a process ...
Karina Cuanalo-Contreras +2 more
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Alpha-Synuclein Oligomers Interact with Metal Ions to Induce Oxidative Stress and Neuronal Death in Parkinson's Disease [PDF]
, 2015 Protein aggregation and oxidative stress are both key pathogenic processes in Parkinson's disease, although the mechanism by which misfolded proteins induce oxidative stress and neuronal death remains unknown.
Andrey Y. Abramov +16 more
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Folding of small disulfide-rich proteins : clarifying the puzzle [PDF]
, 2006 Premi a l'excel·lència investigadora. Àmbit de les Ciències Experimentals. 2008The process by which small proteins fold to their native conformations has been intensively studied over the last few decades.
Apuy +73 more
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Co-Chaperones in Targeting and Delivery of Misfolded Proteins to the 26S Proteasome
Biomolecules, 2020 Protein homeostasis (proteostasis) is essential for the cell and is maintained by a highly conserved protein quality control (PQC) system, which triages newly synthesized, mislocalized and misfolded proteins.
Amanda B. Abildgaard +6 more
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