mRNP granules: assembly, function, and connections with disease

JR Buchan - RNA biology, 2014 - Taylor & Francis
Messenger ribonucleoprotein (mRNP) granules are dynamic, self-assembling structures that
harbor non-translating mRNAs bound by various proteins that regulate mRNA translation …

Nearly 30 years of animal models to study amyotrophic lateral sclerosis: a historical overview and future perspectives

T Bonifacino, RA Zerbo, M Balbi, C Torazza… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell
autonomous neurodegenerative disease characterized by upper and lower motor neuron …

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

CC Chou, YI Zhang, ME Umoh, SW Vaughan… - Nature …, 2018 - nature.com
The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43)
is a common histopathological hallmark of the amyotrophic lateral sclerosis and …

PIKFYVE inhibition mitigates disease in models of diverse forms of ALS

ST Hung, GR Linares, WH Chang, Y Eoh, G Krishnan… - Cell, 2023 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that results from
many diverse genetic causes. Although therapeutics specifically targeting known causal …

Functional significance of TDP-43 mutations in disease

E Buratti - Advances in genetics, 2015 - Elsevier
At present, there are very few therapeutic options for patients affected by amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD). However, almost all patients affected by …

Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALS

L Krug, N Chatterjee, R Borges-Monroy, S Hearn… - PLoS …, 2017 - journals.plos.org
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are two
incurable neurodegenerative disorders that exist on a symptomological spectrum and share …

SQuIRE reveals locus-specific regulation of interspersed repeat expression

WR Yang, D Ardeljan, CN Pacyna… - Nucleic acids …, 2019 - academic.oup.com
Transposable elements (TEs) are interspersed repeat sequences that make up much of the
human genome. Their expression has been implicated in development and disease …

Glial cells in amyotrophic lateral sclerosis

T Philips, JD Rothstein - Experimental neurology, 2014 - Elsevier
For more than twenty years glial cells have been implicated in the pathogenetic cascades for
genetic and sporadic forms of ALS. The biological role of glia, including the principal CNS …

TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

Y Khalfallah, R Kuta, C Grasmuck, A Prat… - Scientific reports, 2018 - nature.com
Stress granules (SGs) are cytoplasmic foci that form in response to various external stimuli
and are essential to cell survival following stress. SGs are studied in several diseases …

Astrocyte physiopathology: at the crossroads of intercellular networking, inflammation and cell death

D Rossi - Progress in neurobiology, 2015 - Elsevier
Recent breakthroughs in neuroscience have led to the awareness that we should revise our
traditional mode of thinking and studying the CNS, ie by isolating the privileged network of …