MitoBloCK-12 (522-51-0) attenuates mitochondrial protein import (4-10 μM). Discovered in a screen of FDA-approved drugs. Blocks translocation of a mutant form of alanine: glyoxylate aminotransferase (AGT) to the mitochondria and restores AGT trafficking to peroxisomes.1 Also reduces oxalate accumulation and thus has potential to treat patients with primary hyperoxaluria 1 who possess mutations in AGT.1 Also displays antimicrobial activity2, inhibits apamin-sensitive K+ channels3 and induces apoptosis by inhibiting XIAP4.
1) Miyata et al. (2014), Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1; Proc. Natl. Acad. Sci. USA 111 14406
2) Frey Tirri et al. (2011), Antimicrobial topical agents used in the vagina; Curr. Probl. Dermatol. 40 36
3) Castle et al. (1993), Dequalinium: a potent inhibitor of apamin-sensitive K+ channels in hepatocytes and of nicotinic responses in skeletal muscle; Eur. J. Pharmacol. 236 201
4) Orzaez et al. (2011), Characterization of dequalinium as a XIAP antagonist that targets the BIR2 domain; Apoptosis 16 460