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Mutant TDP-43 drives impairments in axonal transport and glycolysis in a mouse stem-cell-derived motor neuron model of amyotrophic lateral sclerosis (ALS)

Emily Carroll
Jakub Scaber
Kevin Talbot
ResearchOpen Access31 Jan 2026
Cell Death & Disease

P: 1-10
Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins

Here, the authors show in human iPSC-derived motor neurons from ALS patients and a TDP-43 mouse model that axonal TDP-43 forms G3BP1 positive RNP condensates, which sequester mRNA of nuclear encoded mitochondrial proteins and decrease local protein synthesis in motor neuron axons and neuromuscular junctions.

Topaz Altman
Ariel Ionescu
Eran Perlson
ResearchOpen Access25 Nov 2021
Nature Communications

Volume: 12, P: 1-17
Mutant GGGGCC RNA prevents YY1 from binding to Fuzzy promoter which stimulates Wnt/β-catenin pathway in C9ALS/FTD

Intronic GGGGCC repeat expansion in the C9orf72 gene causes ALS/FTD. Here the authors show that mutant GGGGCC RNA triggers YY1-Fuzzy transcriptional dysregulation which subsequently induces Wnt/β-catenin pathway and activates cell death in C9ALS/FTD.

Zhefan Stephen Chen
Mingxi Ou
Ho Yin Edwin Chan
ResearchOpen Access18 Dec 2023
Nature Communications

Volume: 14, P: 1-19
C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9

The role of microglia in amyotrophic lateral sclerosis (ALS) is unclear. Here, the authors show that iPSC microglia from C9orf72-ALS patients are toxic to motor neurons and identify microglial MMP9 as a potential therapeutic target.

Björn F. Vahsen
Sumedha Nalluru
Kevin Talbot
ResearchOpen Access22 Sept 2023
Nature Communications

Volume: 14, P: 1-16
Human iPSC co-culture model to investigate the interaction between microglia and motor neurons

Björn F. Vahsen
Elizabeth Gray
Kevin Talbot
ResearchOpen Access23 Jul 2022
Scientific Reports

Volume: 12, P: 1-17

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Mutant TDP-43 drives impairments in axonal transport and glycolysis in a mouse stem-cell-derived motor neuron model of amyotrophic lateral sclerosis (ALS)

Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins

Mutant GGGGCC RNA prevents YY1 from binding to Fuzzy promoter which stimulates Wnt/β-catenin pathway in C9ALS/FTD

C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9

Human iPSC co-culture model to investigate the interaction between microglia and motor neurons

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