Reversible protein assemblies in the proteostasis network in health and disease

V Kohler, C Andréasson - Frontiers in Molecular Biosciences, 2023 - frontiersin.org
While proteins populating their native conformations constitute the functional entities of cells,
protein aggregates are traditionally associated with cellular dysfunction, stress and disease …

A mutational atlas for Parkin proteostasis

L Clausen, V Voutsinos, M Cagiada… - Nature …, 2024 - nature.com
Proteostasis can be disturbed by mutations affecting folding and stability of the encoded
protein. An example is the ubiquitin ligase Parkin, where gene variants result in autosomal …

[HTML][HTML] J-domain proteins: From molecular mechanisms to diseases

J Marszalek, P De Los Rios, D Cyr, MP Mayer… - Cell Stress and …, 2024 - Elsevier
J-domain proteins (JDPs) are the largest family of chaperones in most organisms, but much
of how they function within the network of other chaperones and protein quality control …

PRKN-linked familial Parkinson's disease: cellular and molecular mechanisms of disease-linked variants

L Clausen, J Okarmus, V Voutsinos, M Meyer… - Cellular and Molecular …, 2024 - Springer
Parkinson's disease (PD) is a common and incurable neurodegenerative disorder that
arises from the loss of dopaminergic neurons in the substantia nigra and is mainly …

Subunits of an E3 Ligase Complex as Degrons for Efficient Degradation of Cytosolic, Nuclear, and Membrane Proteins

A Verbič, T Lebar, A Praznik, R Jerala - ACS Synthetic Biology, 2024 - ACS Publications
Protein degradation is a highly regulated cellular process crucial to enable the high dynamic
range of the response to external and internal stimuli and to balance protein biosynthesis to …

C-terminal sequence stability profiling in Saccharomyces cerevisiae reveals protective protein quality control pathways

S Hasenjäger, A Bologna, LO Essen… - Journal of Biological …, 2023 - ASBMB
Protein quality control (PQC) mechanisms are essential for degradation of misfolded or
dysfunctional proteins. An essential part of protein homeostasis is recognition of defective …

Importance of an N-terminal structural switch in the distinction between small RNA-bound and free ARGONAUTE

S Bressendorff, IMZ Sjøgaard, A Prestel… - Nature Structural & …, 2025 - nature.com
ARGONAUTE (AGO) proteins bind to small non-coding RNAs to form RNA-induced
silencing complexes. In the RNA-bound state, AGO is stable while RNA-free AGO turns over …

Deep mutational scanning reveals a correlation between degradation and toxicity of thousands of aspartoacylase variants

M Grønbæk-Thygesen, V Voutsinos… - Nature …, 2024 - nature.com
Unstable proteins are prone to form non-native interactions with other proteins and thereby
may become toxic. To mitigate this, destabilized proteins are targeted by the protein quality …

Proteostasis perturbation of N-Myc leveraging HSP70 mediated protein turnover improves treatment of neuroendocrine prostate cancer

P Xu, JC Yang, B Chen, S Ning, X Zhang… - Nature …, 2024 - nature.com
N-Myc is a key driver of neuroblastoma and neuroendocrine prostate cancer (NEPC). One
potential way to circumvent the challenge of undruggable N-Myc is to target the protein …

Systematic characterization of indel variants using a yeast-based protein folding sensor

S Larsen-Ledet, S Lindemose, A Panfilova, S Gersing… - Structure, 2024 - cell.com
Gene variants resulting in insertions or deletions of amino acid residues (indels) have
important consequences for evolution and are often linked to disease, yet, compared to …