Effects of in vivo conditions on amyloid aggregation
One of the grand challenges of biophysical chemistry is to understand the principles that
govern protein misfolding and aggregation, which is a highly complex process that is …
govern protein misfolding and aggregation, which is a highly complex process that is …
Proteomics approaches for biomarker and drug target discovery in ALS and FTD
Neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS) and
frontotemporal dementia (FTD) are increasing in prevalence but lack targeted therapeutics …
frontotemporal dementia (FTD) are increasing in prevalence but lack targeted therapeutics …
Susceptibility of Mutant SOD1 to Form a Destabilized Monomer Predicts Cellular Aggregation and Toxicity but Not In vitro Aggregation Propensity
L McAlary, JA Aquilina, JJ Yerbury - Frontiers in neuroscience, 2016 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
the rapid and progressive degeneration of upper and lower motor neurons in the spinal …
the rapid and progressive degeneration of upper and lower motor neurons in the spinal …
[HTML][HTML] Small heat shock proteins: multifaceted proteins with important implications for life
S Carra, S Alberti, JLP Benesch, W Boelens… - Cell Stress and …, 2019 - Elsevier
Abstract Small Heat Shock Proteins (sHSPs) evolved early in the history of life; they are
present in archaea, bacteria, and eukaryota. sHSPs belong to the superfamily of molecular …
present in archaea, bacteria, and eukaryota. sHSPs belong to the superfamily of molecular …
Amyotrophic lateral sclerosis: proteins, proteostasis, prions, and promises
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of the
motor neurons that innervate muscle, resulting in gradual paralysis and culminating in the …
motor neurons that innervate muscle, resulting in gradual paralysis and culminating in the …
BAG3 Pro209 mutants associated with myopathy and neuropathy relocate chaperones of the CASA-complex to aggresomes
E Adriaenssens, B Tedesco, L Mediani… - Scientific Reports, 2020 - nature.com
Three missense mutations targeting the same proline 209 (Pro209) codon in the co-
chaperone Bcl2-associated athanogene 3 (BAG3) have been reported to cause distal …
chaperone Bcl2-associated athanogene 3 (BAG3) have been reported to cause distal …
Clusterin protects neurons against intracellular proteotoxicity
It is now widely accepted in the field that the normally secreted chaperone clusterin is
redirected to the cytosol during endoplasmic reticulum (ER) stress, although the …
redirected to the cytosol during endoplasmic reticulum (ER) stress, although the …
Most myopathic lamin variants aggregate: A functional genomics approach for assessing variants of uncertain significance
CL Anderson, ER Langer, TC Routes… - NPJ Genomic …, 2021 - nature.com
Hundreds of LMNA variants have been associated with several distinct disease phenotypes.
However, genotype–phenotype relationships remain largely undefined and the impact for …
However, genotype–phenotype relationships remain largely undefined and the impact for …
Unbiased Label-Free Quantitative Proteomics of Cells Expressing Amyotrophic Lateral Sclerosis (ALS) Mutations in CCNF Reveals Activation of the Apoptosis …
The past decade has seen a rapid acceleration in the discovery of new genetic causes of
ALS, with more than 20 putative ALS-causing genes now cited. These genes encode …
ALS, with more than 20 putative ALS-causing genes now cited. These genes encode …
DNAJB chaperones suppress destabilised protein aggregation via a region distinct from that used to inhibit amyloidogenesis
S McMahon, S Bergink, HH Kampinga… - Journal of Cell …, 2021 - journals.biologists.com
Disturbances to protein homeostasis (proteostasis) can lead to protein aggregation and
inclusion formation, processes associated with a variety of neurodegenerative disorders …
inclusion formation, processes associated with a variety of neurodegenerative disorders …