Non-cell-autonomous pathogenic mechanisms in amyotrophic lateral sclerosis

ACM Van Harten, H Phatnani, S Przedborski - Trends in neurosciences, 2021 - cell.com
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset paralytic disorder,
characterized mainly by a loss of motor neurons (MNs) in the CNS. Over the past decades …

The recurrent architecture of tumour initiation, progression and drug sensitivity

A Califano, MJ Alvarez - Nature Reviews Cancer, 2017 - nature.com
Recent studies across multiple tumour types are starting to reveal a recurrent regulatory
architecture in which genomic alterations cluster upstream of functional master regulator …

[HTML][HTML] Excessive release of inorganic polyphosphate by ALS/FTD astrocytes causes non-cell-autonomous toxicity to motoneurons

C Arredondo, C Cefaliello, A Dyrda, N Jury, P Martinez… - Neuron, 2022 - cell.com
Non-cell-autonomous mechanisms contribute to neurodegenerative diseases such as
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), in which astrocytes …

[HTML][HTML] Quantitative assessment of protein activity in orphan tissues and single cells using the metaVIPER algorithm

H Ding, EF Douglass Jr, AM Sonabend, A Mela… - Nature …, 2018 - nature.com
We and others have shown that transition and maintenance of biological states is controlled
by master regulator proteins, which can be inferred by interrogating tissue-specific …

[HTML][HTML] A neuroprotective astrocyte state is induced by neuronal signal EphB1 but fails in ALS models

GE Tyzack, CE Hall, CR Sibley, T Cymes… - Nature …, 2017 - nature.com
Astrocyte responses to neuronal injury may be beneficial or detrimental to neuronal
recovery, but the mechanisms that determine these different responses are poorly …

[HTML][HTML] Rbfox splicing factors promote neuronal maturation and axon initial segment assembly

M Jacko, SM Weyn-Vanhentenryck, JW Smerdon… - Neuron, 2018 - cell.com
Neuronal maturation requires dramatic morphological and functional changes, but the
molecular mechanisms governing this process are not well understood. Here, we studied …

Neurodegeneration: From cellular concepts to clinical applications

A Katsnelson, B De Strooper, HY Zoghbi - Science translational …, 2016 - science.org
Developing therapies for neurodegenerative diseases will require new scientific approaches
that take into account the detrimental effects of altered protein and RNA homeostasis on …

[HTML][HTML] Astrocytes: dissecting their diverse roles in amyotrophic lateral sclerosis and frontotemporal dementia

CF Valori, C Sulmona, L Brambilla, D Rossi - Cells, 2023 - mdpi.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal
neurodegenerative disorders often co-occurring in the same patient, a feature that suggests …

[HTML][HTML] Dysregulation of translation in TDP-43 proteinopathies: deficits in the RNA supply chain and local protein production

RT Bjork, NP Mortimore, S Loganathan… - Frontiers in …, 2022 - frontiersin.org
Local control of gene expression provides critical mechanisms for regulating development,
maintenance and plasticity in the nervous system. Among the strategies known to govern …

TDP-43 enhances translation of specific mRNAs linked to neurodegenerative disease

N Neelagandan, G Gonnella, S Dang… - Nucleic acids …, 2019 - academic.oup.com
The RNA-binding protein TDP-43 is heavily implicated in neurodegenerative disease.
Numerous patient mutations in TARDBP, the gene encoding TDP-43, combined with data …