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 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: 0.514)

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 Protein (TDP-43)protein TARDBP — TAR DNA Binding Protein 43gene TARDBP/TDP-43 Proteinprotein TARDBP Mutations in Amyotrophic Lateral Sclerosisdisease TDP-43 Proteinopathy Neurons in Frontotemporal Demcell Amyotrophic Lateral Sclerosisredirect TDP-43 Pathology in Corticobasal Syndromedisease ALS-FTD-Parkinsonism Comparison Matrixdisease TDP-43 Proteinopathy Neuronscell ALS-FTD Spectrumdisease TDP-43 Proteinopathy Neuronsredirect ALS Progression Rate Heterogeneitydisease TDP-43 Aggregate Neuronscell ALS Pipelinecompany ALS Pipeline Companiescompany

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 (7)

Cross-Seeding Prevention Strategy0.689

Glycine-Rich Domain Competitive Inhibition0.640

Cryptic Exon Silencing Restoration0.531

RNA-Binding Competition Therapy for TDP-43 Cross-Seeding0.465

Glial Neuroinflammatory Amplification by TDP-43 Pathology0.680

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