• Stealth BioTherapeutics Corp (NASDAQ: MITO) presented new SBT-272 preclinical data demonstrating functional improvement in upper motor neurons with TDP-43 pathology.

• TDP-43 pathology plays a significant role in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).

• The data were presented at the Keystone Neurodegeneration Symposium.

• A Phase 1 study to evaluate the safety and tolerability of SBT-272 in healthy volunteers is underway.

• Read Next: Stealth Bio Posts Disappointing Elamipretide Data From Mid-Stage Geographic Atrophy Study.

• The data demonstrated that SBT-272 significantly improved mitochondrial structural integrity and motility in TDP-43 mutant (A315T)-expressing upper motor neurons.

• These enhancements in mitochondrial health were associated with improved axon outgrowth, a key indicator of improved neuronal health.

• The effect of SBT-272 on axon outgrowth was superior to edaravone (approved for the treatment of ALS) and Amylyx Pharmaceuticals Inc (NASDAQ: AMLX) AMX0035 (under FDA review).

• Chronic in vivo administration of SBT-272 reduced upper motor neuron degeneration and neuroinflammation in the motor cortex of the prp‐hTDP‐43A315T‐UeGFP mouse model of ALS.

• Price Action: MITO shares are up 6.60% at $0.27 on the last check Wednesday.

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