SCARB2/LIMPII Recombinant Rabbit mAb (S-2847-21)

SCARB2/LIMPII, also called lysosomal integral membrane protein-2 (LIMP-2), is an ~85 kDa heavily glycosylated type-III multi-pass membrane protein that resides predominantly in the limiting membranes of lysosomes and late endosomes in every mammalian tissue, where it functions as the mannose-6-phosphate-independent trafficking receptor that escorts the lipid-catabolizing enzyme β-glucocerebrosidase (GBA) from the Golgi to the lysosome lumen, and also serves as the primary cell-surface receptor for enterovirus 71 and several coxsackieviruses that cause hand-foot-and-mouth disease, while genetic loss-of-function mutations in SCARB2 produce the autosomal-recessive “action myoclonus–renal failure syndrome” characterized by progressive myoclonic epilepsy, renal glomerulosclerosis, deafness and peripheral neuropathy.