Molecular mechanisms underlying nucleotide repeat expansion disorders

I Malik, CP Kelley, ET Wang, PK Todd - Nature reviews Molecular cell …, 2021 - nature.com
The human genome contains over one million short tandem repeats. Expansion of a subset
of these repeat tracts underlies over fifty human disorders, including common genetic …

Heat shock proteins as immunomodulants

T Zininga, L Ramatsui, A Shonhai - Molecules, 2018 - mdpi.com
Heat shock proteins (Hsps) are conserved molecules whose main role is to facilitate folding
of other proteins. Most Hsps are generally stress-inducible as they play a particularly …

Neurodegenerative disease and the NLRP3 inflammasome

JA Holbrook, HH Jarosz-Griffiths, E Caseley… - Frontiers in …, 2021 - frontiersin.org
The prevalence of neurodegenerative disease has increased significantly in recent years,
and with a rapidly aging global population, this trend is expected to continue. These …

RNA controls PolyQ protein phase transitions

H Zhang, S Elbaum-Garfinkle, EM Langdon, N Taylor… - Molecular cell, 2015 - cell.com
Compartmentalization in cells is central to the spatial and temporal control of biochemistry.
In addition to membrane-bound organelles, membrane-less compartments form partitions in …

Autophagy in neurodegenerative diseases: pathogenesis and therapy

F Guo, X Liu, H Cai, W Le - Brain pathology, 2018 - Wiley Online Library
The most prevalent pathological features of many neurodegenerative diseases are the
aggregation of misfolded proteins and the loss of certain neuronal populations. Autophagy …

The Hsp70/Hsp90 chaperone machinery in neurodegenerative diseases

RE Lackie, A Maciejewski, VG Ostapchenko… - Frontiers in …, 2017 - frontiersin.org
The accumulation of misfolded proteins in the human brain is one of the critical features of
many neurodegenerative diseases, including Alzheimer's disease (AD). Assembles of beta …

The protective role of exercise against age-related neurodegeneration

A Sujkowski, L Hong, RJ Wessells, SV Todi - Ageing Research Reviews, 2022 - Elsevier
Endurance exercise is a widely accessible, low-cost intervention with a variety of benefits to
multiple organ systems. Exercise improves multiple indices of physical performance and …

Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies

A Ciechanover, YT Kwon - Experimental & molecular medicine, 2015 - nature.com
Mammalian cells remove misfolded proteins using various proteolytic systems, including the
ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and …

Targeting RNA Foci in iPSC-Derived Motor Neurons from ALS Patients with a C9ORF72 Repeat Expansion

D Sareen, JG O'Rourke, P Meera… - Science translational …, 2013 - science.org
Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative condition characterized
by loss of motor neurons in the brain and spinal cord. Expansions of a hexanucleotide …

[HTML][HTML] CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome

PK Todd, SY Oh, A Krans, F He, C Sellier, M Frazer… - Neuron, 2013 - cell.com
Fragile X-associated tremor ataxia syndrome (FXTAS) results from a CGG repeat expansion
in the 5′ UTR of FMR1. This repeat is thought to elicit toxicity as RNA, yet disease brains …