TDP-43 mutant mouse model cGAS/STING pathway analysis

Validation Score: 0.900 Price: $0.50 amyotrophic lateral sclerosis TDP-43 mutant mice Status: proposed

What This Experiment Tests

Validation experiment designed to validate causal mechanisms targeting TARDBP in TDP-43 mutant mice. Primary outcome: neuroinflammatory cytokine profile

Description

Investigation of cGAS/STING pathway activation in TDP-43 mutant mice to validate the role of this innate immune signaling cascade in ALS" class="entity-link entity-disease" title="disease: ALS">ALS pathology in vivo. The study examined whether pharmacologic inhibition or genetic deletion of cGAS and STING components could prevent the neuroinflammatory phenotype associated with TDP-43 mutations in the mouse model.

TARGET GENE

TARDBP (Score: 0.510)

MODEL SYSTEM

TDP-43 mutant mice

ESTIMATED COST

TIMELINE

0 months

PATHWAY

cGAS/STING innate immune signaling

SOURCE

extracted_from_pmid_33031745

PRIMARY OUTCOME

neuroinflammatory cytokine profile

Scoring Dimensions

📖 Wiki Pages

TARDBP/TDP-43 Proteinprotein TARDBP Protein (TDP-43)protein TARDBP — TAR DNA Binding Protein 43gene TARDBP Mutations in Amyotrophic Lateral Sclerosisdisease STING Inhibitor Therapy in Neurodegenerationtherapeutic ALS Treatment Strategiestherapeutic ALS Treatment Overviewtherapeutic ALS Therapeuticstherapeutic ALS Therapeutic Landscape — Programs by Phase and disease ALS Cure Roadmaptherapeutic ALS Communication Brain-Computer Interfacestechnology TDP-43 Proteinredirect TDP-43 Proteinprotein TDP-43 Proteinprotein TDP-43 Proteinredirect

Protocol

Analysis of TDP-43 mutant mice with pharmacologic inhibition or genetic deletion of cGAS/STING components, assessment of inflammatory markers and pathology

Expected Outcomes

cGAS/STING inhibition reduces neuroinflammation in TDP-43 mutant mice

Success Criteria

Decreased inflammatory cytokine levels and reduced pathology

Related Hypotheses (4)

Cryptic Exon Silencing Restoration0.462

Cross-Seeding Prevention Strategy0.451

Glycine-Rich Domain Competitive Inhibition0.429

RNA-Binding Competition Therapy for TDP-43 Cross-Seeding0.374

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