Sample Type | n | Range | Average |
---|---|---|---|
Serum | 5 | 85% - 101% | 93% |
EDTA Plasma | 5 | 93% - 104% | 97% |
Heparin Plasma | 5 | 86% - 105% | 97% |
Sample Type | n | 1:2 | 1:4 | 1:8 |
---|---|---|---|---|
Serum | 5 | 92-104% | 85-102% | 85-105% |
EDTA Plasma | 5 | 85-96% | 83-97% | 93-101% |
Heparin Plasma | 5 | 80-96% | 81-94% | 83-94% |
Item | Quantity | Storage |
---|---|---|
Pre-Coated 96 Well Microplate | 12 x 8 Well Strips | +4°C |
Lyopholized Standard | 2 Vials | +4°C |
Sample Dilution Buffer | 20ml | +4°C |
Biotinylated Detection Antibody | 120µl | +4°C |
Antibody Dilution Buffer | 10ml | +4°C |
HRP-Streptavidin Conjugate | 120µl | +4°C |
SABC Dilution Buffer | 10ml | +4°C |
TMB Substrate | 10ml | +4°C |
Stop Solution | 10ml | +4°C |
Wash Buffer (25X) | 30ml | +4°C |
Plate Sealers | 5 Adhesive Strips | - |
Foil Pouch | 1 Zip-Sealed Pouch | - |
Neuroinflammation exacerbates the progression of SOD1-driven amyotrophic lateral sclerosis (ALS), although the underlying mechanisms remain largely unknown. Herein, we demonstrate that misfolded SOD1 (SOD1Mut)-causing ALS results in mitochondrial damage, thus triggering the release of mtDNA and an RNA:DNA hybrid into the cytosol in an mPTP-independent manner to activate IRF3- and IFNAR-dependent type I interferon (IFN-I) and interferon-stimulating genes. The neuronal hyper-IFN-I and pro-inflammatory responses triggered in ALS-SOD1Mut were sufficiently robust to cause a strong physiological outcome in vitro and in vivo. cGAS/DDX41-STING-signaling is amplified in bystander cells through inter-neuronal gap junctions. Our results highlight the importance of a common DNA-sensing pathway between SOD1 and TDP-43 in influencing the progression of ALS.