Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?

A Marcelo, R Koppenol, LP de Almeida, CA Matos… - Cell death & …, 2021 - nature.com
Stress granules (SGs) are membraneless cell compartments formed in response to different
stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other …

RNA-binding proteins with prion-like domains in health and disease

AF Harrison, J Shorter - Biochemical Journal, 2017 - portlandpress.com
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD).
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …

Huntington's disease: mechanisms of pathogenesis and therapeutic strategies

M Jimenez-Sanchez, F Licitra… - Cold Spring …, 2017 - perspectivesinmedicine.cshlp.org
Huntington's disease is a late-onset neurodegenerative disease caused by a CAG
trinucleotide repeat in the gene encoding the huntingtin protein. Despite its well-defined …

A chaperome subnetwork safeguards proteostasis in aging and neurodegenerative disease

M Brehme, C Voisine, T Rolland, S Wachi, JH Soper… - Cell reports, 2014 - cell.com
Chaperones are central to the proteostasis network (PN) and safeguard the proteome from
misfolding, aggregation, and proteotoxicity. We categorized the human chaperome of 332 …

The life cycle of the 26S proteasome: from birth, through regulation and function, and onto its death

I Livneh, V Cohen-Kaplan, C Cohen-Rosenzweig… - Cell research, 2016 - nature.com
The 26S proteasome is a large,∼ 2.5 MDa, multi-catalytic ATP-dependent protease complex
that serves as the degrading arm of the ubiquitin system, which is the major pathway for …

Drosophila as an In Vivo Model for Human Neurodegenerative Disease

L McGurk, A Berson, NM Bonini - Genetics, 2015 - academic.oup.com
With the increase in the ageing population, neurodegenerative disease is devastating to
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …

Cellular strategies for controlling protein aggregation

J Tyedmers, A Mogk, B Bukau - Nature reviews Molecular cell biology, 2010 - nature.com
The aggregation of misfolded proteins is associated with the perturbation of cellular function,
ageing and various human disorders. Mounting evidence suggests that protein aggregation …

Unfolding the role of protein misfolding in neurodegenerative diseases

C Soto - Nature Reviews Neuroscience, 2003 - nature.com
Recent evidence indicates that diverse neurodegenerative diseases might have a common
cause and pathological mechanism—the misfolding, aggregation and accumulation of …

Polyglutamine (PolyQ) Diseases: Navigating the Landscape of Neurodegeneration

R Tenchov, JM Sasso, QA Zhou - ACS Chemical Neuroscience, 2024 - ACS Publications
Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …

Impairment of the ubiquitin-proteasome system by protein aggregation

NF Bence, RM Sampat, RR Kopito - Science, 2001 - science.org
Intracellular deposition of aggregated and ubiquitylated proteins is a prominent
cytopathological feature of most neurodegenerative disorders. Whether protein aggregates …