Anti-LBPA Antibody, clone 6C4 clone 6C4, from mouse

Lyso bis-phosphatidic acid (LBPA, Lysobisphosphatidic acid, L(bis)PA) is a lipid that is exclusively located in late endosomes and is used as a late endosome marker. One of the characteristic features of late endosomes is a complex system of internal membranes within the lumen that contains large amounts of the unique, poorly degradable LBPA, and thus forms a specialized membrane domain within endosomes. Late endosomes function not only as a major protein-sorting compartment, but also an obligatory station for LDL and other endocytosed ligands destined for degradation. Research shows that the LBPA-rich membranes within late endosomes regulate cholesterol transport, presumably by acting as a collection and distribution device. The genetic disease Niemann-Pick type C (NPC) is characterized by both lysosomal and endosomal storage disorders. In skin fibroblasts from NPC patients, cholesterol accumulation is seen within vesicles containing late endosome markers LBPA and Rab7.