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Molecular Mechanisms of Disease in ALS & FTD

Molecular Mechanisms of Disease in ALS & FTD diagram by

Mutations in RNA-binding proteins, including TARDBP, have been found in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). This feed focuses on molecular mechanisms that underlie ALS and FTD.

Top 20 most recent papers
bioRxiv

TDP-43 dysfunction restricts dendritic complexity by inhibiting CREB activation and altering gene expression

bioRxivDecember 13, 2019
Josiah J. HerzogSuzanne Paradis
2
Brain : a Journal of Neurology

Homozygous GRN mutations: new phenotypes and new insights into pathological and molecular mechanisms

Brain : a Journal of NeurologyDecember 20, 2019
Vincent HuinIsabelle Le Ber
15
1
Journal of Neurology, Neurosurgery, and Psychiatry

Cross-sectional and longitudinal measures of chitinase proteins in amyotrophic lateral sclerosis and expression of CHI3L1 in activated astrocytes

Journal of Neurology, Neurosurgery, and PsychiatryJanuary 16, 2020
Lucas VuRobert Bowser
3
1
Cells

Mitophagy in Alzheimer's Disease and Other Age-Related Neurodegenerative Diseases

CellsJanuary 16, 2020
Qian Cai, Yu Young Jeong
2
1
bioRxiv

Genetic ablation of SOD1G37R selectively from corticofugal projection neurons protects corticospinal neurons from degeneration without affecting ALS onset and progression

bioRxivJanuary 10, 2020
Jelena Scekic-ZahirovicCaroline Rouaux
3
The European Journal of Neuroscience

The influence of metallothionein treatment and treadmill running exercise on disease onset and survival in SOD1G93A amyotrophic lateral sclerosis mice

The European Journal of NeuroscienceJanuary 19, 2020
Katherine Ea LewisMeng Inn Chuah
bioRxiv

Variant-specific changes in persistent or resurgent Na+ current in SCN8A-EIEE13 iPSC-derived neurons

bioRxivJanuary 18, 2020
Andrew M TidballJack M Parent
Journal of Neurology

ALS-derived fibroblasts exhibit reduced proliferation rate, cytoplasmic TDP-43 aggregation and a higher susceptibility to DNA damage

Journal of NeurologyJanuary 16, 2020
Javier RianchoMiguel Lafarga
1
Cells

Hippocampal Neurogenesis Is Enhanced in Adult Tau Deficient Mice

CellsJanuary 18, 2020
Marangelie Criado-MarreroAnd Laura J Blair
International Journal of Biological Sciences

Nicotinamide Riboside Enhances Mitochondrial Proteostasis and Adult Neurogenesis through Activation of Mitochondrial Unfolded Protein Response Signaling in the Brain of ALS SOD1G93A Mice

International Journal of Biological SciencesJanuary 14, 2020
Qi ZhouRenshi Xu
1
Cellular and Molecular Life Sciences : CMLS

Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stress

Cellular and Molecular Life Sciences : CMLSDecember 6, 2019
Mark HalloranJulie D Atkin
6
1
International Journal of Biological Macromolecules

Computational insights into mechanism of AIM4-mediated inhibition of aggregation of TDP-43 protein implicated in ALS and evidence for in vitro inhibition of liquid-liquid phase separation (LLPS) of TDP-432C -A315T by AIM4

International Journal of Biological MacromoleculesJanuary 10, 2020
Amandeep GirdharBasant K Patel
1
ELife

Relaxation of synaptic inhibitory events as a compensatory mechanism in fetal SOD spinal motor networks

ELifeDecember 24, 2019
Pascal BranchereauDaniel Cattaert
1
1
Ageing Research Reviews

Does perturbation in the mitochondrial protein folding pave the way for neurodegeneration diseases?

Ageing Research ReviewsDecember 10, 2019
Ting JiYang Yang
7
1
Bioorganic & Medicinal Chemistry

Expanding therapeutic opportunities for neurodegenerative diseases: A perspective on the important role of phenotypic screening

Bioorganic & Medicinal ChemistryJanuary 1, 2020
Susanne E Swalley
2
1
Neurobiology of Disease

Longitudinal transcriptomic analysis of altered pathways in a CHMP2Bintron5 -based model of ALS-FTD

Neurobiology of DiseaseDecember 15, 2019
Waegaert RobinRené Frédérique
2
1
International Neurourology Journal

Exercise and Neuroinflammation in Health and Disease

International Neurourology JournalDecember 5, 2019
Dae-Yun SeoHyo-Bum Kwak
1
Brain : a Journal of Neurology

Decoding the relationship between ageing and amyotrophic lateral sclerosis: a cellular perspective

Brain : a Journal of NeurologyDecember 19, 2019
Virenkumar A Pandya, Rickie Patani
47
1
Frontiers in Pharmacology

Epigenetic Regulation of Excitatory Amino Acid Transporter 2 in Neurological Disorders

Frontiers in PharmacologyJanuary 11, 2020
Mohammad Afaque Alam, Prasun K Datta
3
1
The Journal of Physical Chemistry. B

Exploring Folding Aspects of Monomeric Superoxide Dismutase

The Journal of Physical Chemistry. BJanuary 4, 2020
Paulo Ricardo MouroJorge Chahine
1

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