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PRKN-linked familial Parkinson's disease: cellular and molecular mechanisms of disease-linked variants
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 …
arises from the loss of dopaminergic neurons in the substantia nigra and is mainly …
A mutational atlas for Parkin proteostasis
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 …
protein. An example is the ubiquitin ligase Parkin, where gene variants result in autosomal …
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 …
may become toxic. To mitigate this, destabilized proteins are targeted by the protein quality …
Reversible protein assemblies in the proteostasis network in health and disease
While proteins populating their native conformations constitute the functional entities of cells,
protein aggregates are traditionally associated with cellular dysfunction, stress and disease …
protein aggregates are traditionally associated with cellular dysfunction, stress and disease …
Proteostasis perturbation of N-Myc leveraging HSP70 mediated protein turnover improves treatment of neuroendocrine prostate cancer
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 …
potential way to circumvent the challenge of undruggable N-Myc is to target the protein …
[HTML][HTML] J-domain proteins: From molecular mechanisms to diseases
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 …
of how they function within the network of other chaperones and protein quality control …
Disease-linked mutations cause exposure of a protein quality control degron
C Kampmeyer, S Larsen-Ledet, MR Wagnkilde… - Structure, 2022 - cell.com
More than half of disease-causing missense variants are thought to lead to protein
degradation, but the molecular mechanism of how these variants are recognized by the cell …
degradation, but the molecular mechanism of how these variants are recognized by the cell …
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 …
range of the response to external and internal stimuli and to balance protein biosynthesis to …
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 …
silencing complexes. In the RNA-bound state, AGO is stable while RNA-free AGO turns over …
[HTML][HTML] Prediction of quality-control degradation signals in yeast proteins
KE Johansson, B Mashahreh… - Journal of Molecular …, 2023 - Elsevier
Effective proteome homeostasis is key to cellular and organismal survival, and cells
therefore contain efficient quality control systems to monitor and remove potentially toxic …
therefore contain efficient quality control systems to monitor and remove potentially toxic …