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 …

The clinical trial landscape in amyotrophic lateral sclerosis—Past, present, and future

HJ Wobst, KL Mack, DG Brown… - Medicinal research …, 2020 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease marked by
progressive loss of muscle function. It is the most common adult‐onset form of motor neuron …

Suppression of mutant C9orf72 expression by a potent mixed backbone antisense oligonucleotide

H Tran, MP Moazami, H Yang, D McKenna-Yasek… - Nature medicine, 2022 - nature.com
Expansions of a G4C2 repeat in the C9ORF72 gene are the most common genetic cause of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), two devastating …

CRISPR/Cas9-mediated excision of ALS/FTD-causing hexanucleotide repeat expansion in C9ORF72 rescues major disease mechanisms in vivo and in vitro

KE Meijboom, A Abdallah, NP Fordham… - Nature …, 2022 - nature.com
A GGGGCC24+ hexanucleotide repeat expansion (HRE) in the C9ORF72 gene is the most
common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia …

Nuclear import receptors directly bind to arginine-rich dipeptide repeat proteins and suppress their pathological interactions

S Hutten, S Usluer, B Bourgeois, F Simonetti, HM Odeh… - Cell reports, 2020 - cell.com
Nuclear import receptors, also called importins, mediate nuclear import of proteins and
chaperone aggregation-prone cargoes (eg, neurodegeneration-linked RNA-binding …

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 …

Stress granule subtypes: an emerging link to neurodegeneration

VM Advani, P Ivanov - Cellular and Molecular Life Sciences, 2020 - Springer
Abstract Stress Granules (SGs) are membraneless cytoplasmic RNA granules, which
contain translationally stalled mRNAs, associated translation initiation factors and multiple …

Proximity proteomics of C9orf72 dipeptide repeat proteins identifies molecular chaperones as modifiers of poly-GA aggregation

F Liu, D Morderer, MC Wren… - Acta Neuropathologica …, 2022 - Springer
The most common inherited cause of two genetically and clinico-pathologically overlapping
neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal …

C9-ALS-associated proline-arginine dipeptide repeat protein induces activation of NLRP3 inflammasome of HMC3 microglia cells by binding of complement …

RH Fu, CW Tsai, SC Chiu, SP Liu, YT Chiang, YH Kuo… - Cells, 2022 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal disease in which motor neurons gradually
degenerate. The mutation of the C9orf72 gene is the main genetic cause of ALS (C9-ALS) …

C9orf72-derived arginine-containing dipeptide repeats associate with axonal transport machinery and impede microtubule-based motility

L Fumagalli, FL Young, S Boeynaems, M De Decker… - Science …, 2021 - science.org
A hexanucleotide repeat expansion in the C9orf72 gene is the most common genetic cause
of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). How this mutation …