BIP-135 (20 μM; 48 hours) is a superior neuroprotective agent in the model of oxidative stress[1]. BIP-135 (20-30 μM; 72 hours) increases the survival motor neuron (SMN) protein levels at a dose of 25 μM in human SMA fibroblasts. And the typical bell-shaped dose-response curve is observed due to some toxicity at higher concentrations[1].

BIP-135 (75 mg/kg; i.p.; daily; from postnatal day 0 to 21) prolongs the median survival time of Δ7 SMA KO mouse model of spinal muscular atrophy. And it does not appear to be toxic and was well-tolerated by the animals (no decrease in body weight)[1].