[HTML][HTML] Targeting the nucleolus as a therapeutic strategy in human disease

A Corman, O Sirozh, V Lafarga… - Trends in Biochemical …, 2023 - cell.com
The nucleolus is the site of ribosome biogenesis, one of the most resource-intensive
processes in eukaryotic cells. Accordingly, nucleolar morphology and activity are highly …

It's getting complicated—a fresh look at p53-MDM2-ARF triangle in tumorigenesis and cancer therapy

CP Kung, JD Weber - Frontiers in cell and developmental biology, 2022 - frontiersin.org
Anti-tumorigenic mechanisms mediated by the tumor suppressor p53, upon oncogenic
stresses, are our bodies' greatest weapons to battle against cancer onset and development …

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 …

Ribosome inhibition by C9ORF72-ALS/FTD-associated poly-PR and poly-GR proteins revealed by cryo-EM

AB Loveland, E Svidritskiy, D Susorov, S Lee… - Nature …, 2022 - nature.com
Toxic dipeptide-repeat (DPR) proteins are produced from expanded G4C2 repeats in the
C9ORF72 gene, the most common genetic cause of amyotrophic lateral sclerosis (ALS) and …

[HTML][HTML] Nucleosomes in mammalian sperm: conveying paternal epigenetic inheritance or subject to reprogramming between generations?

L Gaspa-Toneu, AHFM Peters - Current Opinion in Genetics & …, 2023 - Elsevier
The genome of mammalian sperm is largely packaged by sperm-specific proteins termed
protamines. The presence of some residual nucleosomes has, however, emerged as a …

The skeletal muscle emerges as a new disease target in amyotrophic lateral sclerosis

O Pikatza-Menoio, A Elicegui, X Bengoetxea… - Journal of personalized …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that leads to
progressive degeneration of motor neurons (MNs) and severe muscle atrophy without …

Nucleolar stress caused by arginine-rich peptides triggers a ribosomopathy and accelerates aging in mice

O Sirozh, A Saez-Mas, B Jung, L Sanchez-Burgos… - Molecular Cell, 2024 - cell.com
Nucleolar stress (NS) has been associated with age-related diseases such as cancer or
neurodegeneration. To investigate how NS triggers toxicity, we used (PR) n arginine-rich …

Reduced C9orf72 expression exacerbates polyGR toxicity in patient iPSC-derived motor neurons and a Type I protein arginine methyltransferase inhibitor reduces …

TL Dane, AL Gill, FG Vieira, KR Denton - Frontiers in Cellular …, 2023 - frontiersin.org
Introduction Intronic repeat expansions in the C9orf72 gene are the most frequent known
single genetic causes of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia …

HNRNPK alleviates RNA toxicity by counteracting DNA damage in C9orf72 ALS

E Braems, V Bercier, E Van Schoor, K Heeren… - Acta …, 2022 - Springer
A 'GGGGCC'repeat expansion in the first intron of the C9orf72 gene is the most common
genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) …

Arg/Lys-containing IDRs are cryptic binding domains for ATP and nucleic acids that interplay to modulate LLPS

M Dang, T Li, S Zhou, J Song - Communications Biology, 2022 - nature.com
Most membrane-less organelles (MLOs) formed by LLPS contain both nucleic acids and IDR-
rich proteins. Currently while IDRs are well-recognized to drive LLPS, nucleic acids are …