Detection and degradation of stalled nascent chains via ribosome-associated quality control

CS Sitron, O Brandman - Annual review of biochemistry, 2020 - annualreviews.org
Stalled protein synthesis produces defective nascent chains that can harm cells. In
response, cells degrade these nascent chains via a process called ribosome-associated …

Oculopharyngeal muscular dystrophy

B Brais, GA Rouleau, JP Bouchard… - Seminars in …, 1999 - thieme-connect.com
Autosomal dominant oculopharyngeal muscular dystrophy (OPMD) is an adult-onset
disease with worldwide distribution. It usually presents in the fifth or sixth decades with …

Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy

JE Davies, S Sarkar… - Human molecular …, 2006 - academic.oup.com
Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease that
presents in the fifth or sixth decade with dysphagia, ptosis and proximal limb weakness …

Alternative low-populated conformations prompt phase transitions in polyalanine repeat expansions

R Antón, MÁ Treviño, D Pantoja-Uceda, S Félix… - Nature …, 2024 - nature.com
Abnormal trinucleotide repeat expansions alter protein conformation causing malfunction
and contribute to a significant number of incurable human diseases. Scarce structural …

Amyloid aggregates arise from amino acid condensations under prebiotic conditions

J Greenwald, MP Friedmann, R Riek - Angewandte Chemie, 2016 - Wiley Online Library
Current theories on the origin of life reveal significant gaps in our understanding of the
mechanisms that allowed simple chemical precursors to coalesce into the complex polymers …

Contributions of NH⊙⊙⊙ O and CH⊙⊙⊙ O Hydrogen Bonds to the Stability of β-Sheets in Proteins

S Scheiner - The Journal of Physical Chemistry B, 2006 - ACS Publications
Ab initio quantum calculations are applied to both the parallel and the antiparallel
arrangements of the β-sheets of proteins. The energies of the NH⊙⊙⊙ O and CH⊙⊙⊙ O …

Structure of core domain of fibril-forming PHF/Tau fragments

H Inouye, D Sharma, WJ Goux, DA Kirschner - Biophysical journal, 2006 - cell.com
Short peptide sequences within the microtubule binding domain of the protein Tau are
proposed to be core nucleation sites for formation of amyloid fibrils displaying the paired …

Diversity of molecular transformations involved in the formation of spider silks

T Lefèvre, S Boudreault, C Cloutier… - Journal of molecular …, 2011 - Elsevier
Spiders that spin orb webs secrete seven types of silk. Although the spinning process of the
dragline thread is beginning to be understood, the molecular events that occur in spiders' …

[HTML][HTML] Oculopharyngeal muscular dystrophy: recent advances in the understanding of the molecular pathogenic mechanisms and treatment strategies

A Abu-Baker, GA Rouleau - … et Biophysica Acta (BBA)-Molecular Basis of …, 2007 - Elsevier
Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disorder characterized by
progressive eyelid drooping, swallowing difficulties and proximal limb weakness. OPMD is …

Toxicity of internalized polyalanine to cells depends on aggregation

Y Iizuka, R Owada, T Kawasaki, F Hayashi… - Scientific reports, 2021 - nature.com
In polyalanine (PA) diseases, the disease-causing transcription factors contain an
expansion of alanine repeats. While aggregated proteins that are responsible for the …