Biological importance of arginine: A comprehensive review of the roles in structure, disorder, and functionality of peptides and proteins

MN Gupta, VN Uversky - International Journal of Biological Macromolecules, 2023 - Elsevier
Abstract Arginine shows Jekyll and Hyde behavior in several respects. It participates in
protein folding via ionic and H-bonds and cation-pi interactions; the charge and …

Messenger RNA translation defects in neurodegenerative diseases

E Storkebaum, K Rosenblum… - New England Journal of …, 2023 - Mass Medical Soc
Messenger RNA Translation Defects in Neurodegenerative Diseases | New England Journal of
Medicine Skip to main content The New England Journal of Medicine homepage Advanced …

CLIP-Seq analysis enables the design of protective ribosomal RNA bait oligonucleotides against C9ORF72 ALS/FTD poly-GR pathophysiology

JA Ortega, IR Sasselli, M Boccitto, AC Fleming… - Science …, 2023 - science.org
Amyotrophic lateral sclerosis and frontotemporal dementia patients with a hexanucleotide
repeat expansion in C9ORF72 (C9-HRE) accumulate poly-GR and poly-PR aggregates. The …

The need for biomarkers in the ALS–FTD spectrum: a clinical point of view on the role of proteomics

F Vignaroli, A Mele, G Tondo, V De Giorgis, M Manfredi… - Proteomes, 2023 - mdpi.com
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are severely
debilitating and progressive neurodegenerative disorders. A distinctive pathological feature …

Transcriptome-wide RNA binding analysis of C9orf72 poly (PR) dipeptides

R Balendra, IR de Los Mozos, HM Odeh… - Life Science …, 2023 - life-science-alliance.org
An intronic GGGGCC repeat expansion in C9orf72 is a common genetic cause of
amyotrophic lateral sclerosis and frontotemporal dementia. The repeats are transcribed in …

Translation dysregulation in neurodegenerative diseases: a focus on ALS

S Wang, S Sun - Molecular neurodegeneration, 2023 - Springer
RNA translation is tightly controlled in eukaryotic cells to regulate gene expression and
maintain proteome homeostasis. RNA binding proteins, translation factors, and cell …

CGG repeat expansion in LRP12 in amyotrophic lateral sclerosis

K Kume, T Kurashige, K Muguruma, H Morino… - The American Journal of …, 2023 - cell.com
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the
degeneration of motor neurons. Although repeat expansion in C9orf72 is its most common …

[HTML][HTML] Aberrant phase separation is a common killing strategy of positively charged peptides in biology and human disease

S Boeynaems, XR Ma, V Yeong, GM Ginell, JH Chen… - bioRxiv, 2023 - ncbi.nlm.nih.gov
Positively charged repeat peptides are emerging as key players in neurodegenerative
diseases. These peptides can perturb diverse cellular pathways but a unifying framework for …

Arginine-rich C9ORF72 ALS proteins stall ribosomes in a manner distinct from a canonical ribosome-associated quality control substrate

V Kriachkov, AR Ormsby, EP Kusnadi… - Journal of Biological …, 2023 - ASBMB
Hexanucleotide expansion mutations in C9ORF72 are a frequent cause of amyotrophic
lateral sclerosis. We previously reported that long arginine-rich dipeptide repeats (DPRs) …

Differential toxicity and localization of arginine-rich C9ORF72 dipeptide repeat proteins depend on de-clustering of positive charges

T Miyagi, K Ueda, M Sugimoto, T Yagi, D Ito… - Iscience, 2023 - cell.com
Arginine-rich dipeptide repeat proteins (R-DPRs), poly (PR) and poly (GR), translated from
the hexanucleotide repeat expansion in the amyotrophic lateral sclerosis (ALS)-causative …