FUS and TDP-43 phases in health and disease

B Portz, BL Lee, J Shorter - Trends in biochemical sciences, 2021 - cell.com
The distinct prion-like domains (PrLDs) of FUS and TDP-43, modulate phase transitions that
result in condensates with a range of material states. These assemblies are implicated in …

Protein assembly systems in natural and synthetic biology

G Chiesa, S Kiriakov, AS Khalil - BMC biology, 2020 - Springer
The traditional view of protein aggregation as being strictly disease-related has been
challenged by many examples of cellular aggregates that regulate beneficial biological …

Engineered protein disaggregases mitigate toxicity of aberrant prion-like fusion proteins underlying sarcoma

JJ Ryan, ML Sprunger, K Holthaus, J Shorter… - Journal of Biological …, 2019 - ASBMB
FUS and EWSR1 are RNA-binding proteins with prion-like domains (PrLDs) that aggregate
in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The FUS and …

The hunt for ancient prions: archaeal prion-like domains form amyloid-based epigenetic elements

T Zajkowski, MD Lee, SS Mondal… - Molecular Biology …, 2021 - academic.oup.com
Prions, proteins that can convert between structurally and functionally distinct states and
serve as non-Mendelian mechanisms of inheritance, were initially discovered and only …

It pays to be in phase

AK Itakura, RA Futia, DF Jarosz - Biochemistry, 2018 - ACS Publications
To survive, organisms must orchestrate competing biochemical and regulatory processes in
time and space. Recent work has suggested that the underlying chemical properties of some …

Molecular origins of complex heritability in natural genotype-to-phenotype relationships

CM Jakobson, DF Jarosz - Cell Systems, 2019 - cell.com
The statistical complexity of heredity has long been evident, but its molecular origins remain
elusive. To investigate, we charted 90 comprehensive genotype-to-phenotype maps in a …

Measuring prion propagation in single bacteria elucidates a mechanism of loss

K Jager, MT Orozco-Hidalgo… - Proceedings of the …, 2023 - National Acad Sciences
Prions are self-propagating protein aggregates formed by specific proteins that can adopt
alternative folds. Prions were discovered as the cause of the fatal transmissible spongiform …

The Celiac‐Disease Superantigen Oligomerizes and Increases Permeability in an Enterocyte Cell Model

MG Herrera, MJ Amundarain… - Angewandte Chemie …, 2024 - Wiley Online Library
Celiac disease (CeD) is an autoimmune disorder triggered by gluten proteins, affecting
approximately 1% of the global population. The 33‐mer deamidated gliadin peptide (DGP) …

IDPs and IDRs in biomolecular condensates

AS Holehouse - Intrinsically disordered proteins, 2019 - Elsevier
Over the last decade, there has been considerable interest in the role of intrinsically
disordered proteins and protein regions in the formation of nonstoichiometric assemblies …

What has a century of quantitative genetics taught us about nature's genetic tool kit?

CM Jakobson, DF Jarosz - Annual Review of Genetics, 2020 - annualreviews.org
The complexity of heredity has been appreciated for decades: Many traits are controlled not
by a single genetic locus but instead by polymorphisms throughout the genome. The …