Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins

A Ratti, E Buratti - Journal of neurochemistry, 2016 - Wiley Online Library
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …

Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects

J Mitra, EN Guerrero, PM Hegde… - Proceedings of the …, 2019 - National Acad Sciences
Genome damage and their defective repair have been etiologically linked to degenerating
neurons in many subtypes of amyotrophic lateral sclerosis (ALS) patients; however, the …

Disruption of RNA metabolism in neurological diseases and emerging therapeutic interventions

JK Nussbacher, R Tabet, GW Yeo, C Lagier-Tourenne - Neuron, 2019 - cell.com
RNA binding proteins are critical to the maintenance of the transcriptome via controlled
regulation of RNA processing and transport. Alterations of these proteins impact multiple …

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 …

Cytoplasmic functions of TDP-43 and FUS and their role in ALS

N Birsa, MP Bentham, P Fratta - Seminars in cell & developmental biology, 2020 - Elsevier
TAR DNA-binding protein of 43 kDa (TDP-43) and fused in sarcoma (FUS) are RNA binding
proteins (RBPs) primarily located in the nucleus, and involved in numerous aspects of RNA …

RNA-binding proteins in neurological development and disease

S Prashad, PP Gopal - RNA biology, 2021 - Taylor & Francis
ABSTRACT RNA-binding proteins are a critical group of multifunctional proteins that
precisely regulate all aspects of gene expression, from alternative splicing to mRNA …

RNA metabolism in neurodegenerative disease

EY Liu, CP Cali, EB Lee - Disease models & mechanisms, 2017 - journals.biologists.com
Aging-related neurodegenerative diseases are progressive and fatal neurological diseases
that are characterized by irreversible neuron loss and gliosis. With a growing population of …

RNA modifications and RNA metabolism in neurological disease pathogenesis

B Chatterjee, CKJ Shen, P Majumder - International journal of molecular …, 2021 - mdpi.com
The intrinsic cellular heterogeneity and molecular complexity of the mammalian nervous
system relies substantially on the dynamic nature and spatiotemporal patterning of gene …

Structural plasticity of the hippocampus in neurodegenerative diseases

PDE Weerasinghe-Mudiyanselage, MJ Ang… - International journal of …, 2022 - mdpi.com
Neuroplasticity is the capacity of neural networks in the brain to alter through development
and rearrangement. It can be classified as structural and functional plasticity. The …