Bis(monoacylglycero)phosphate (BMP) (BMP)

Bis(monoacylglycero)phosphate (BMP), also known as lysobisphosphatidic acid (LBPA), is a unique anionic phospholipid localized almost exclusively to the internal membranes of late endosomes and lysosomes (Kobayashi et al., 1998). It possesses an unusual sn-1:sn-1' stereoconfiguration, which confers high resistance to the acidic environment and degradative enzymes of the lysosome (Hullin-Matsuda et al., 2007). BMP serves as an essential docking platform and cofactor for sphingolipid activator proteins (saposins), which are required for the degradation of membrane lipids by lysosomal hydrolases (Kolter & Sandhoff, 2005). Beyond lipid catabolism, BMP is crucial for regulating cholesterol homeostasis by facilitating the efflux of cholesterol from lysosomes via NPC}

Other names

Lysobisphosphatidic acidLBPAsn-glycero-3-phospho-1'-sn-glycerolBis-monoacyl-glyceryl-phosphate

Arimoclomol acts as a heat shock protein (HSP) co-inducer, specifically increasing levels of HSP70. HSP70 then translocates to the lysosome where it binds to Bis(monoacylglycero)phosphate (BMP) in a pH-dependent manner. This binding stabilizes the lysosomal membrane and promotes the recruitment and activity of lysosomal enzymes, such as acid sphingomyelinase, thereby facilitating the degradation of accumulated lipids (Kirkegaard et al., 2010; Schuchman et al., 2021).