APC and amyotrophic lateral sclerosis: Subsequently, in 2014, Zlokovic and colleagues showed that BSCB breakdown contributed to early motor neuron degeneration in ALS mice, and that restoring BSCB integrity via treatment with coagulation-deficient engineered variants of APC (5A-APC; RR230/231AA and KKK192-194AAA) early in the disease also slowed motor neuron degeneration [112].