TDP-43/FUS in motor neuron disease: Complexity and challenges

EN Guerrero, H Wang, J Mitra, PM Hegde… - Progress in …, 2016 - Elsevier
Amyotrophic lateral sclerosis (ALS), a common motor neuron disease affecting two per
100,000 people worldwide, encompasses at least five distinct pathological subtypes …

[HTML][HTML] TDP-43 and inflammation: implications for amyotrophic lateral sclerosis and frontotemporal dementia

F Bright, G Chan, A van Hummel, LM Ittner… - International Journal of …, 2021 - mdpi.com
The abnormal mislocalisation and ubiquitinated protein aggregation of the TAR DNA
binding protein 43 (TDP-43) within the cytoplasm of neurons and glia in the central nervous …

[HTML][HTML] Dysregulated microRNAs in amyotrophic lateral sclerosis microglia modulate genes linked to neuroinflammation

C Parisi, I Arisi, N D'Ambrosi, AE Storti, R Brandi… - Cell death & …, 2013 - nature.com
MicroRNAs (miRNAs) regulate gene expression at post-transcriptional level and are key
modulators of immune system, whose dysfunction contributes to the progression of …

Connecting TDP-43 pathology with neuropathy

JR Klim, G Pintacuda, LA Nash, IG San Juan… - Trends in …, 2021 - cell.com
Transactive response DNA-binding protein 43 kDa (TDP-43), a multifunctional nucleic acid-
binding protein, is a primary component of insoluble aggregates associated with several …

SQSTM1 mutations–bridging Paget disease of bone and ALS/FTLD

SL Rea, V Majcher, MS Searle, R Layfield - Experimental cell research, 2014 - Elsevier
Paget disease of bone (PDB) is a skeletal disorder common in Western Europe but
extremely rare in the Indian subcontinent and Far East. The condition has a strong genetic …

[HTML][HTML] NF-κB-induced IL-6 ensures STAT3 activation and tumor aggressiveness in glioblastoma

BC McFarland, SW Hong, R Rajbhandari, GB Twitty Jr… - PloS one, 2013 - journals.plos.org
Glioblastoma (GBM) is the most aggressive, neurologically destructive and deadly tumor of
the central nervous system (CNS). In GBM, the transcription factors NF-κB and STAT3 are …

Astrocytes expressing ALS‐linked mutant FUS induce motor neuron death through release of tumor necrosis factor‐alpha

A Kia, K McAvoy, K Krishnamurthy, D Trotti, P Pasinelli - Glia, 2018 - Wiley Online Library
Mutations in fused in sarcoma (FUS) are linked to amyotrophic lateral sclerosis (ALS), a fatal
neurodegenerative disease affecting both upper and lower motor neurons. While it is …

[HTML][HTML] MicroRNA-125b regulates microglia activation and motor neuron death in ALS

C Parisi, G Napoli, S Amadio, A Spalloni… - Cell Death & …, 2016 - nature.com
Understanding the means by which microglia self-regulate the neuroinflammatory response
helps modulating their reaction during neurodegeneration. In amyotrophic lateral sclerosis …

[HTML][HTML] The established and emerging roles of astrocytes and microglia in amyotrophic lateral sclerosis and frontotemporal dementia

RA Radford, M Morsch, SL Rayner, NJ Cole… - Frontiers in cellular …, 2015 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two progressive,
fatal neurodegenerative syndromes with considerable clinical, genetic and pathological …

Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic

R Mancuso, X Navarro - Progress in Neurobiology, 2015 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by
progressive degeneration of upper and lower motoneurons, leading to muscle weakness …