Mechanisms and pathology of protein misfolding and aggregation

N Louros, J Schymkowitz, F Rousseau - Nature Reviews Molecular Cell …, 2023 - nature.com
Despite advances in machine learning-based protein structure prediction, we are still far
from fully understanding how proteins fold into their native conformation. The conventional …

Structure–function relationships in protein homorepeats

CA Elena-Real, P Mier, N Sibille… - Current Opinion in …, 2023 - Elsevier
Homorepeats (or polyX), protein segments containing repetitions of the same amino acid,
are abundant in proteomes from all kingdoms of life and are involved in crucial biological …

[HTML][HTML] Pathologic polyglutamine aggregation begins with a self-poisoning polymer crystal

T Kandola, S Venkatesan, J Zhang, BT Lerbakken… - Elife, 2023 - elifesciences.org
A long-standing goal of amyloid research has been to characterize the structural basis of the
rate-determining nucleating event. However, the ephemeral nature of nucleation has made …

[HTML][HTML] Mechanism and evolutionary origins of alanine-tail C-degron recognition by E3 ligases Pirh2 and CRL2-KLHDC10

PR Patil, AM Burroughs, M Misra, F Cerullo, CC Insua… - Cell reports, 2023 - cell.com
In ribosome-associated quality control (RQC), nascent polypeptides produced by interrupted
translation are modified with C-terminal polyalanine tails (" Ala-tails") that function outside …

[HTML][HTML] The structural plasticity of polyglutamine repeats

PJB Pereira, JA Manso, S Macedo-Ribeiro - Current Opinion in Structural …, 2023 - Elsevier
From yeast to humans, polyglutamine (polyQ) repeat tracts are found frequently in the
proteome and are particularly prominent in the activation domains of transcription factors …

Site-specific introduction of alanines for the nuclear magnetic resonance investigation of low-complexity regions and large biomolecular assemblies

CA Elena-Real, A Urbanek, L Imbert… - ACS Chemical …, 2023 - ACS Publications
Nuclear magnetic resonance (NMR) studies of large biomolecular machines and highly
repetitive proteins remain challenging due to the difficulty of assigning frequencies to …

Polyglutamine disease proteins: Commonalities and differences in interaction profiles and pathological effects

M Bonsor, O Ammar, S Schnoegl, EE Wanker… - …, 2024 - Wiley Online Library
Currently, nine polyglutamine (polyQ) expansion diseases are known. They include
spinocerebellar ataxias (SCA1, 2, 3, 6, 7, 17), spinal and bulbar muscular atrophy (SBMA) …

The sequence context in poly-alanine regions: structure, function and conservation

P Mier, CA Elena-Real, J Cortés, P Bernadó… - …, 2022 - academic.oup.com
Motivation Poly-alanine (polyA) regions are protein stretches mostly composed of alanines.
Despite their abundance in eukaryotic proteomes and their association to nine inherited …

[HTML][HTML] A glutamine-based single α-helix scaffold to target globular proteins

A Escobedo, J Piccirillo, J Aranda, T Diercks… - Nature …, 2022 - nature.com
The binding of intrinsically disordered proteins to globular ones can require the folding of
motifs into α-helices. These interactions offer opportunities for therapeutic intervention but …

[HTML][HTML] Coaggregation of polyglutamine (polyQ) proteins is mediated by polyQ-tract interactions and impairs cellular proteostasis: Coaggregation of polyQ proteins

JY Hong, JY Wang, HW Yue, XL Zhang… - Acta Biochimica et …, 2023 - ncbi.nlm.nih.gov
Nine polyglutamine (polyQ) proteins have already been identified that are considered to be
associated with the pathologies of neurodegenerative disorders called polyQ diseases, but …