Cystatin B

Cystatin B, or stefin B, is a ubiquitously expressed type 1 intracellular cystatin and one of the major cytosolic inhibitors of cathepsins B, H, and L, dampening protease activity that leaks from lysosomes. Beyond protease inhibition, it interacts with cellular partners involved in apoptosis and has been proposed to modulate caspase activity. Expansions of a dodecamer repeat in the CSTB promoter cause progressive myoclonus epilepsy of Unverricht-Lundborg type (EPM1), linking cystatin B directly to neurodegeneration. Recombinant cystatin B serves studies of cathepsin regulation, EPM1 disease mechanisms, and neuronal proteostasis. Unlike type 2 cystatins, cystatin B lacks glycosylation and functions chiefly in the cytosol.