Graphene quantum dots modulate stress granule assembly and prevent abnormal phase transition of fused in sarcoma protein

H Zhang, J Gu, Y Zhang, H Guo, S Zhang, J Song… - ACS …, 2023 - ACS Publications
Protein liquid–liquid phase separation (LLPS) plays a crucial role in mediating dynamic
assembly of different membraneless organelles such as stress granules (SGs) …

[HTML][HTML] Genetic mutations in RNA-binding proteins and their roles in ALS

K Kapeli, FJ Martinez, GW Yeo - Human genetics, 2017 - Springer
Mutations in genes that encode RNA-binding proteins (RBPs) have emerged as critical
determinants of neurological diseases, especially motor neuron disorders such as …

The role of FUS gene variants in neurodegenerative diseases

H Deng, K Gao, J Jankovic - Nature Reviews Neurology, 2014 - nature.com
The neurodegenerative diseases are a diverse group of disorders characterized by
progressive loss of specific groups of neurons. These diseases affect different populations …

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

L Ferraiuolo, J Kirby, AJ Grierson, M Sendtner… - Nature Reviews …, 2011 - nature.com
Amyotrophic lateral sclerosis (ALS) is a genetically diverse disease. At least 15 ALS-
associated gene loci have so far been identified, and the causative gene is known in …

Bridging biophysics and neurology: aberrant phase transitions in neurodegenerative disease

NB Nedelsky, JP Taylor - Nature Reviews Neurology, 2019 - nature.com
Biomolecular condensation arising through phase transitions has emerged as an essential
organizational strategy that governs many aspects of cell biology. In particular, the role of …

[HTML][HTML] Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

S Boeynaems, E Bogaert, E Michiels, I Gijselinck… - Scientific reports, 2016 - nature.com
Hexanucleotide repeat expansions in C9orf72 are the most common cause of amyotrophic
lateral sclerosis (ALS) and frontotemporal degeneration (FTD)(c9ALS/FTD). Unconventional …

Are aberrant phase transitions a driver of cellular aging?

S Alberti, AA Hyman - BioEssays, 2016 - Wiley Online Library
Why do cells age? Recent advances show that the cytoplasm is organized into many
membrane‐less compartments via a process known as phase separation, which ensures …

[HTML][HTML] RNA dysregulation in amyotrophic lateral sclerosis

Z Butti, SA Patten - Frontiers in genetics, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease
and is characterized by the degeneration of upper and lower motor neurons. It has become …

The genetics and neuropathology of amyotrophic lateral sclerosis

A Al-Chalabi, A Jones, C Troakes, A King… - Acta …, 2012 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of motor neurons
leading to death from respiratory failure within about 3 years of symptom onset. A family …

Advances in understanding the molecular basis of frontotemporal dementia

R Rademakers, M Neumann… - Nature Reviews Neurology, 2012 - nature.com
Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular
basis. Until recently, the underlying cause was known in only a minority of cases that were …