Judith Frydman

The paper provides a comprehensive survey of the ubiquitin-proteasome system (UPS) and estimates its size and scope within the human proteostasis network.

Q-DOAS detects early protein aggregation events in real time using a proximity-quenching assay, providing quantitative data for studying neurodegenerative diseases.

This study shows TRiC/CCT regulates RNA polymerase II activity in the nucleus, affecting RNA homeostasis by modulating nascent RNA production.

The paper introduces UbSeRP to map co-translational ubiquitination across the translatome, revealing RQC substrates, biophysical features predicting RQC, and aging-related changes in CTU.

This study identifies NAC's role in regulating translation elongation, cotranslational folding, and organelle targeting through sequence-specific interactions with nascent polypeptides.

This study shows that BDNF and TRiC-inspired reagents can rescue synaptic deficits in HD mouse models.

The paper shows that aging in killifish brains reduces basic amino acid-rich proteins through translation pausing, not transcription or proteasome issues, revealing a vulnerability in nucleic-acid protein biogenesis.

The paper curates a list of 838 high-confidence components of the autophagy-lysosome pathway as part of a comprehensive effort to define the human proteostasis network.

This study uses cryoET and proteomics to identify mitochondrial and organelle abnormalities in HD models, showing potential for early diagnosis and therapy development.

A fragment-based computational method designs nanobodies targeting specific epitopes with nanomolar affinities, without requiring high-resolution antigen structures.

The paper uses nanoLuciferase to report on SARS-CoV2 and VLP production, finding it is packaged and secreted but not with Dengue or Zika.

The paper defines and annotates 959 genes comprising the human proteostasis network, focusing on translation, protein folding, and organelle-specific systems.

This study identifies ribotoxic collisions on CAG expansions as a mechanism causing proteostasis disruption and stress responses in Huntington's Disease.

This study uses cryo-electron tomography to analyze the structure of mutant huntingtin exon 1 and polyQ aggregates, revealing structural heterogeneity and a 'polyQ core' model.

This study shows that neural stem cell differentiation rewires the chaperone network, reducing TRiC/CCT and increasing sHSPs, which shifts proteostasis strategy and may contribute to neurodegenerative disease susceptibility.