LAMP2

Function

The protein expressed by gene LAMP2 plays an important role in chaperone-mediated autophagy, facilitating the lysosomal degradation of proteins in response to various stresses and as part of normal protein turnover, especially for those with a long biological half-life. It functions by binding target proteins like GAPDH and MLLT11, directing them for lysosomal degradation, and is necessary for the fusion of autophagosomes with lysosomes during autophagy. Although cells lacking LAMP2 express normal VAMP8 levels, they fail to accumulate STX17 on autophagosomes, likely explaining the fusion failure between autophagosomes and lysosomes. LAMP2 is essential for the normal degradation of autophagosomal contents and efficient MHCII-mediated presentation of exogenous antigens, as it facilitates lysosomal protein degradation. It is not required for the presentation of endogenous antigens via MHCII. Specifically, isoform LAMP-2C modulates chaperone-mediated autophagy, reduces presentation of endogenous antigens by MHCII, and does not influence the presentation of exogenous and membrane-derived antigens by MHCII. This supplementary information is collated from multiple sources and compiled automatically.

Involvement in disease

Danon disease

DAND

DAND is a lysosomal glycogen storage disease characterized by the clinical triad of cardiomyopathy, vacuolar myopathy and intellectual disability. It is often associated with an accumulation of glycogen in muscle and lysosomes.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

O- and N-glycosylated; some of the 16 N-linked glycans are polylactosaminoglycans.

Sequence Similarities

Belongs to the LAMP family.

Tissue Specificity

Isoform LAMP-2A is highly expressed in placenta, lung and liver, less in kidney and pancreas, low in brain and skeletal muscle (PubMed:26856698, PubMed:7488019). Isoform LAMP-2B is detected in spleen, thymus, prostate, testis, small intestine, colon, skeletal muscle, brain, placenta, lung, kidney, ovary and pancreas and liver (PubMed:26856698, PubMed:7488019). Isoform LAMP-2C is detected in small intestine, colon, heart, brain, skeletal muscle, and at lower levels in kidney and placenta (PubMed:26856698).

Cellular localization

• Lysosome membrane
• Single-pass type I membrane protein
• Endosome membrane
• Single-pass type I membrane protein
• Cell membrane
• Single-pass type I membrane protein
• Cytoplasmic vesicle
• Autophagosome membrane
• This protein shuttles between lysosomes, endosomes, and the plasma membrane.

Alternative names

CD107b, Lysosome-associated membrane glycoprotein 2, LAMP-2, Lysosome-associated membrane protein 2, CD107 antigen-like family member B, LGP-96, LAMP2

Database links

swissprot:P13473 entrezGene:3920 omim:309060

Other research areas

• Immuno-oncology
• Oncology