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amyotrophic lateral sclerosis

Human Sensorimotor Organoids Derived from Healthy and Amyotrophic Lateral Sclerosis Stem Cells Form Neuromuscular Junctions

[Nature Communications] Investigators generated sensorimotor organoids containing physiologically functional neuromuscular junctions and applied the model to different subgroups of amyotrophic lateral sclerosis.

Transcriptome Analysis Using Patient iPSC-Derived Skeletal Myocytes: Bet1L as a New Molecule Possibly Linked to Neuromuscular Degeneration in ALS

[Experimental Neurology] To study common disease mechanisms in amyotrophic lateral sclerosis (ALS) skeletal muscle, the authors performed RNA sequencing of skeletal myocytes differentiated from induced pluripotent stem cells derived from familial ALS and sporadic ALS patients.

Current Progress in the Creation, Characterization, and Application of Human Stem Cell-Derived In Vitro Neuromuscular Junction Models

[Stem Cell Reviews and Reports] The authors outline the current progress towards fully PSC-derived in vitro co-culture models of the human neuromuscular junction and discuss future directions and challenges that must be overcome in order to create reproducible fully PSC-derived models that can be used for developmental studies, disease modeling, and drug testing.

Cedars-Sinai Awarded $11.99 Million for ALS Clinical Trial

[Cedars-Sinai] Cedars-Sinai has been awarded $11.99 million by California's stem cell agency to launch a clinical trial testing a potential gene and stem cell therapy for amyotrophic lateral sclerosis (ALS).

Resolving Cell State in iPSC-Derived Human Neural Samples with Multiplexed Fluorescence Imaging

[Communications Biology] Researchers applied PRobe-based Imaging for Sequential Multiplexing (PRISM) to facilitate multiplexed imaging with facile, rapid exchange of imaging probes to analyze iPSC-derived cortical and motor neuron cultures that were relevant to psychiatric and neurodegenerative disease models, using over ten protein targets.

TMEM106B Modifies TDP-43 Pathology in Human ALS Brain and Cell-Based Models of TDP-43 Proteinopathy

[Acta Neuropathologica] Researchers investigated the influence of genotypes at TMEM106B, a locus associated with risk for frontotemporal lobar degeneration with TAR DNA-binding protein-43 inclusions (FTLD-TDP), and hexanucleotide repeat expansions in C9orf72, a known genetic cause for both amyotrophic lateral sclerosis and FTLD-TDP, on global TDP-43 pathology and regional distribution of TDP-43 pathology in 899 postmortem cases from a spectrum of neurodegenerative diseases.

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