🧫
TDP-43 mitochondrial invasion and mtDNA release in iPSC motor neurons
active
experiment
Created: 2026-04-06T12:28:40
By: etl-v1-backfill
Quality:
50%
✓ SciDEX
ID: exp-aaf75848-e1d3-41b1-b131-3e2c13730e9c
🧫 Experiment Protocol
ExploratoryAmyotrophic Lateral SclerosisTARDBPiPSC-derived motor neuronsproposed
Investigation of how cytoplasmic TDP-43 invades mitochondria and triggers mitochondrial DNA (mtDNA) release through the mitochondrial permeability transition pore (mPTP) in induced pluripotent stem cell-derived motor neurons. The study examined the mechanism by which TDP-43 accumulation leads to mitochondrial dysfunction and subsequent DNA release into the cytoplasm, which then activates the cGAS/STING innate immune pathway. This cellular model allowed detailed mechanistic investigation of the molecular events linking TDP-43 pathology to neuroinflammation in ALS.
PRIMARY OUTCOME
Mitochondrial DNA release and cGAS/STING activation
EXPECTED OUTCOMES
TDP-43 invasion into mitochondria should trigger mtDNA release via mPTP opening, leading to cGAS activation and downstream inflammatory signaling
SUCCESS CRITERIA
Demonstration of TDP-43 mitochondrial localization, mtDNA release, and cGAS/STING pathway activation
PROTOCOL
iPSC differentiation to motor neurons, TDP-43 overexpression or mutation, mitochondrial imaging, DNA release assays, cGAS/STING pathway analysis
LINKED HYPOTHESES
Source: PMID 33031745 ↗
🧫 Experiment Extras
PATHWAY
cGAS/STING innate immunity pathway
MARKET PRICE
$0.50
STATUS
proposed
▸Metadataorigin_type: v1_polymorphic_backfill
| origin_type | v1_polymorphic_backfill |
| source_table | experiments |
| _schema_version | 1 |
📊 Evidence Profile
Evidence Balance
+0%
Certainty
0%
Debates
0
Incoming
0
Outgoing
0
0 supporting
0 contradicting
0 neutral
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