L. Plate

Proteostasis Landscapes of Cystic Fibrosis Variants Reveals Drug Response Vulnerability

The paper uses proteomics and computational modeling to analyze CFTR variants, identifying structural vulnerabilities in NBD1 that limit corrector efficacy and suggesting secondary mutations as a therapeutic strategy.

The glycoprotein quality control factor Malectin promotes coronavirus replication and viral protein biogenesis

This study identifies Malectin as a host factor that promotes coronavirus replication and viral protein biogenesis by interacting with viral nonstructural proteins.

The proteostasis interactomes of trafficking-deficient KV11.1 variants associated with Long QT Syndrome and pharmacological chaperone rescue

The paper identifies proteostasis interactomes of trafficking-deficient KV11.1 variants and shows pharmacological chaperone rescue of proteostasis network alterations in LQTS-associated variants.

Site-Specific Crosslinking Reveals Phosphofructokinase-L Inhibition Drives Self-Assembly and Attenuation of Protein Interactions

The paper identifies PFKL interactors at specific interfaces and shows compartmentalization reduces interactions at interface 2.

The unfolded protein response transcription factor XBP1s ameliorates Alzheimer`s disease by improving synaptic function and proteostasis

This study shows that overexpressing the active form of XBP1 in neurons reduces amyloid deposits and improves synaptic function in Alzheimer's disease models.

Ribosomal Frameshifting Selectively Modulates the Assembly, Function, and Pharmacological Rescue of a Misfolded CFTR Variant

This paper identifies an RNA structure in the CFTR transcript that promotes ribosomal frameshifting and premature termination, enhancing the function and pharmacological rescue of the misfolded {Delta}F508 CFTR variant.

The endoplasmic reticulum stress sensor IRE1 regulates collagen secretion through the enforcement of the proteostasis factor P4HB/PDIA1 contributing to liver damage and fibrosis

This study shows that IRE1 regulates collagen secretion via P4HB, contributing to liver fibrosis in models of CCl4 and high fat diet.

SARS-CoV-2 Nonstructural Proteins 3 and 4 tune the Unfolded Protein Response

This study investigates how SARS-CoV-2 nonstructural proteins 3 and 4 modulate the Unfolded Protein Response (UPR) through proteomic analysis, revealing their distinct roles in activating specific UPR branches.

Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics

This study investigates how Elexacaftor/VX-445 affects CFTR interactome remodeling in response to specific mutations, revealing differential correction mechanisms.

Elucidation of Global Trends in the Effects of VX-661 and VX-445 on the Expression of Clinical CFTR Variants

This study compares the effects of VX-661 and VX-445 on 129 CFTR variants using deep mutational scanning, finding distinct patterns of expression rescue based on variant destabilization and binding pocket locations.

Time-Resolved Interactome Profiling Deconvolutes Secretory Protein Quality Control Dynamics

This study develops TRIP, a mass-spectrometry method to map protein interactions over time, and applies it to study hypothyroidism by analyzing thyroglobulin interactions and identifying VCP and TEX264 as key factors in protein degradation.

Identification of replication fork-associated proteins in Drosophila embryos and cultured cells using iPOND coupled to quantitative mass spectrometry

The paper identifies replication fork-associated proteins in Drosophila embryos and cultured cells using iPOND and mass spectrometry, finding BRWD3 promotes fork progression and genome stability.

Distinct proteostasis states drive pharmacologic chaperone susceptibility for Cystic Fibrosis Transmembrane Conductance Regulator misfolding mutants

The paper investigates why different CFTR mutations respond variably to the pharmacological chaperone VX-809 by analyzing proteostasis interactions and degradation pathways.

A Family of Glycosylated Macrolides Selectively Target Energetic Vulnerabilities in Leukemia

This study identifies the mitochondrial ATP synthase as the target of apoptolidin family glycomacrolides and demonstrates their efficacy in suppressing leukemia progression.

Comparative host interactomes of the SARS-CoV-2 nonstructural protein 3 and human coronavirus homologs

This study compares the host interactomes of SARS-CoV-2 nsp3 and its coronavirus homologs to identify differences in host interactions that may contribute to pathogenicity.

Glycosylation Limits Forward Trafficking of the Tetraspan Membrane Protein PMP22

The paper shows that N-linked glycosylation limits PMP22 cell surface trafficking and identifies key quality control factors in its biogenesis.

Comparative multiplexed interactomics of SARS-CoV-2 and homologous coronavirus non-structural proteins identifies unique and shared host-cell dependencies

This study compares the interactomes of SARS-CoV-1, SARS-CoV-2, and hCoV-OC43 non-structural proteins to identify unique and shared host-cell dependencies, revealing potential therapeutic targets.

Dengue and Zika virus infection are impaired by small molecule ER proteostasis regulator 147 in an ATF6-independent manner

This study shows that compound 147 reduces Dengue and Zika virus infection by targeting ER proteostasis without relying on ATF6 activation.

Thyroglobulin Interactome Profiling Uncovers Molecular Mechanisms of Thyroid Dyshormonogenesis

The paper identifies proteostasis network interactions affecting Tg processing and reveals mutation-specific changes in N-glycosylation components for degradation.

Deconvoluting Stress-Responsive Proteostasis Signaling Pathways for Pharmacologic Activation using Targeted RNA-sequencing

The paper develops a targeted RNA-seq assay to screen and deconvolute stress-responsive signaling pathway activation, demonstrating its utility for identifying selective pharmacologic activators.

Quantitative Interactome Proteomics Reveals a Molecular Basis for ATF6-Dependent Regulation of a Destabilized Amyloidogenic Protein

The paper identifies how ATF6 activation reduces secretion of a destabilized amyloidogenic protein by enhancing its retention in the ER through specific proteostasis factors.