RNA drugs and RNA targets for small molecules: principles, progress, and challenges

AM Yu, YH Choi, MJ Tu, RM TOUYZ - Pharmacological reviews, 2020 - Elsevier
RNA-based therapies, including RNA molecules as drugs and RNA-targeted small
molecules, offer unique opportunities to expand the range of therapeutic targets. Various …

On the wrong DNA track: Molecular mechanisms of repeat-mediated genome instability

AN Khristich, SM Mirkin - Journal of Biological Chemistry, 2020 - ASBMB
Expansions of simple tandem repeats are responsible for almost 50 human diseases, the
majority of which are severe, degenerative, and not currently treatable or preventable. In this …

CGG repeat RNA G-quadruplexes interact with FMRpolyG to cause neuronal dysfunction in fragile X-related tremor/ataxia syndrome

S Asamitsu, Y Yabuki, S Ikenoshita, K Kawakubo… - Science …, 2021 - science.org
Fragile X-related tremor/ataxia syndrome (FXTAS) is a neurodegenerative disease caused
by CGG triplet repeat expansions in FMR1, which elicit repeat-associated non-AUG (RAN) …

The integrated stress response and phosphorylated eukaryotic initiation factor 2α in neurodegeneration

S Bond, C Lopez-Lloreda, PJ Gannon… - … of Neuropathology & …, 2020 - academic.oup.com
The proposed molecular mechanisms underlying neurodegenerative pathogenesis are
varied, precluding the development of effective therapies for these increasingly prevalent …

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 …

Trinucleotide CGG repeat diseases: an expanding field of polyglycine proteins?

M Boivin, N Charlet-Berguerand - Frontiers in Genetics, 2022 - frontiersin.org
Microsatellites are repeated DNA sequences of 3–6 nucleotides highly variable in length
and sequence and that have important roles in genomes regulation and evolution. However …

C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation

NV Licata, R Cristofani, S Salomonsson… - The EMBO …, 2022 - embopress.org
Abstract Intronic GGGGCC (G4C2) hexanucleotide repeat expansion within the human
C9orf72 gene represents the most common cause of familial forms of amyotrophic lateral …

A 5′ UTR GGN repeat controls localisation and translation of a potassium leak channel mRNA through G-quadruplex formation

CJ Maltby, JPR Schofield, SD Houghton… - Nucleic Acids …, 2020 - academic.oup.com
Abstract RNA G-quadruplexes (G4s) are secondary structures proposed to function as
regulators of post-transcriptional mRNA localisation and translation. G4s within some …

Drug screen in iPSC-Neurons identifies nucleoside analogs as inhibitors of (G4C2) n expression in C9orf72 ALS/FTD

M Czuppa, A Dhingra, Q Zhou, C Schludi, L König… - Cell reports, 2022 - cell.com
Summary An intronic (G 4 C 2) n expansion in C9orf72 causes amyotrophic lateral sclerosis
and frontotemporal dementia primarily through gain-of-function mechanisms: the …

Advancements in clinical RNA therapeutics: Present developments and prospective outlooks

PE Saw, E Song - Cell Reports Medicine, 2024 - cell.com
RNA molecules have emerged as promising clinical therapeutics due to their ability to
target" undruggable" proteins or molecules with high precision and minimal side effects …