Lysosome-associated membrane glycoprotein 2 isoform B (LAMP2B)

Lysosome-associated membrane glycoprotein 2 isoform B (LAMP2B) is a type I integral membrane glycoprotein predominantly found in lysosomes, where it is essential for the fusion of autophagic vacuoles (autophagosomes) with lysosomes, particularly in cardiac and skeletal muscle and to a lesser degree in the brain[1][2][3][4][6]. It differs from other LAMP2 isoforms (LAMP2A and LAMP2C) by its distinct C-terminal sequence, resulting from alternative splicing[1][3][4]. LAMP2B interacts with ATG14 and VAMP8 via its cytosolic coiled-coil domain to promote autophagosome–lysosome fusion[4], a function critical for cellular quality control via macroautophagy. Mutations or deficiencies in LAMP2B are causative for Danon disease, a severe X-linked disorder characterized by cardiomyopathy, skeletal myopathy, and sometimes intellectual disability[2][4]. Current therapeutic strategies center on gene correction or restoration of LAMP2B function, though it is not yet a direct target of approved drugs. The protein is also a minor component of exosome membranes, suggesting roles in intercellular communication[3]. There is emerging evidence for its involvement in certain cancers and possibly neurodegenerative diseases, reflecting its central function in cellular degradation pathways and lysosomal health[3][4].

Other names

CD107bMac-3LAMP-2BLAMP-2 (generic gene/protein, with "B" specifying the isoform)LAMP2_HUMANLGP110

Not directly a drug target yet; therapeutic concepts involve gene correction or restoration of LAMP2B-mediated autophagic fusion