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Description

Lysosome-associated membrane glycoprotein 2 (UniProt: P13473, also known as LAMP-2, Lysosome-associated membrane protein 2, CD107 antigen-like family member B, LGP-96, CD107b) is encoded by the LAMP2 gene (Gene ID: 3920) in human. LAMP-2 is a single-pass type I, heavily glycosylated, membrane protein found in plasma membrane, endosomes, and lysosomes. It is synthesized with a signal peptide (aa 1-28), which is subsequently cleaved off to generate the mature form that contains a lumenal domain (aa 29-375), a transmembrane domain (aa 376-399), and a short cytoplasmic tail (aa 400-410). It is an important component of lysosomal membranes and is involved in both autophagy and lysosomal membrane permeabilization. LAMP-2 plays an important role in chaperone-mediated autophagy. It is required for the fusion of autophagosomes with lysosomes during autophagy. It also plays a role in lysosomal protein degradation in response to starvation. It functions by binding target proteins, such as GAPDH and MLLT11, and targets them for lysosomal degradation. In cancer cells, chronic acidosis-induced upregulation of LAMP-2 has been reported that protects these cells from acid-induced hydrolysis and promotes their survival via chaperone-mediated autophagy. Loss of LAMP-2 is reported to aggravate oxidative stress through the obstruction of reactive oxygen species clearance. Three different isoforms of LAMP-2 have been described that are produced by alternative splicing. Mutations in LAMP2 gene have been linked to Danon disease that is characterized by cardiomyopathy, vascular myopathy, and mental retardation. It is often associated with accumulation of glycogen in muscle and lysosomes.