Results 21 to 30 of about 53,939 (158)

Highly efficient protein misfolding cyclic amplification. [PDF]

open access: yesPLoS Pathogens, 2011
Protein misfolding cyclic amplification (PMCA) provides faithful replication of mammalian prions in vitro and has numerous applications in prion research.
Nuria Gonzalez-Montalban   +6 more
doaj   +1 more source

Therapeutic Targeting of Proteostasis in Amyotrophic Lateral Sclerosis—a Systematic Review and Meta-Analysis of Preclinical Research

open access: yesFrontiers in Neuroscience, 2020
Background: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive fatal neurodegenerative condition. There are no effective treatments. The only globally licensed medication, that prolongs life by 2–3 months, was approved by the FDA in 1995.
Elizabeth Elliott   +27 more
doaj   +1 more source

Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative disease. [PDF]

open access: yesPLoS Biology, 2010
Neurodegenerative diseases such as Huntington disease are devastating disorders with no therapeutic approaches to ameliorate the underlying protein misfolding defect inherent to poly-glutamine (polyQ) proteins.
Daniel W Neef   +2 more
doaj   +1 more source

Effect of spermidine on misfolding and interactions of alpha-synuclein. [PDF]

open access: yesPLoS ONE, 2012
Alpha-synuclein (α-Syn) is a 140 aa presynaptic protein which belongs to a group of natively unfolded proteins that are unstructured in aqueous solutions.
Alexey V Krasnoslobodtsev   +5 more
doaj   +1 more source

Impact of translational error‐induced and error‐free misfolding on the rate of protein evolution

open access: yesMolecular Systems Biology, 2010
What determines the rate of protein evolution is a fundamental question in biology. Recent genomic studies revealed a surprisingly strong anticorrelation between the expression level of a protein and its rate of sequence evolution.
Jian‐Rong Yang   +2 more
doaj   +1 more source

Divergent effects of PERK and IRE1 signaling on cell viability.

open access: yesPLoS ONE, 2009
Protein misfolding in the endoplasmic reticulum (ER) activates a set of intracellular signaling pathways, collectively termed the Unfolded Protein Response (UPR). UPR signaling promotes cell survival by reducing misfolded protein levels.
Jonathan H Lin   +4 more
doaj   +1 more source

Matrin3: Disorder and ALS Pathogenesis

open access: yesFrontiers in Molecular Biosciences, 2022
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the degeneration of both upper and lower motor neurons in the brain and spinal cord.
Ahmed Salem   +7 more
doaj   +1 more source

Bridging the gap: From protein misfolding to protein misfolding diseases

open access: yesFEBS 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
openaire   +2 more sources

Theoretical model of prion propagation: A misfolded protein induces misfolding [PDF]

open access: yesProceedings of the National Academy of Sciences, 2005
There is a hypothesis that dangerous diseases such as bovine spongiform encephalopathy, Creutzfeldt-Jakob, Alzheimer's, fatal familial insomnia, and several others are induced by propagation of wrong or misfolded conformations of some vital proteins. If for some reason the misfolded conformations were acquired by many such protein molecules it might ...
Edyta, Małolepsza   +3 more
openaire   +2 more sources

Stress and viral insults do not trigger E200K PrP conversion in human cerebral organoids.

open access: yesPLoS ONE, 2022
Prion diseases are a group of rare, transmissible, and invariably fatal neurodegenerative diseases that affect both humans and animals. The cause of these diseases is misfolding of the prion protein into pathological isoforms called prions.
Anna Smith   +9 more
doaj   +1 more source

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