LAMP-2/CD107b Antibody (H4B4) [mFluor Violet 500 SE]

mFluor(TM) is a trademark of AAT Bioquest, Inc. This conjugate is made on demand. Actual recovery may vary from the stated volume of this product. The volume will be greater than or equal to the unit size stated on the datasheet.

LAMP-2 (Lysosome-associated membrane protein 2) is a single-pass type I membrane protein that belongs to a family of membrane glycoproteins (~40 KDa). LAMP-2 protein is encoded by nine exons, with the first 8 exons and a portion of exon 9 encoding the highly glycosylated protein domains within the lysosomal lumen. The transmembrane and cytosolic carboxy-terminal domains of LAMP-2 are encoded by the remaining sequence of exon 9 and conform the receptor for targeting proteins to the lysosome. Splicing of exon 9 in the LAMP-2 pre mRNA leads to various splice forms with distinct cytosolic domains. Three splice variants, LAMP-2A, -2B and -2C, have been identified which shuttle between the plasma membrane, endosomal compartment and lysosomes (1). Tissue specific expression has been described for each LAMP-2 splice variant, with LAMP-2A being more ubiquitously expressed (e.g., placenta, lung, liver, pancreas and prostate), LAMP-2B predominantly expressed in skeletal muscle and LAMP-2C in brain tissue (1). All LAMP-2 splice variants participate in lysosomal degradation processes. LAMP-2A is the only variant that serves as a receptor targeting proteins for lysosomal degradation in chaperone-mediated autophagy (2,3). LAMP-2B is essential for macroautophagy in cardiomyocytes, where it facilitates autophagosome-lysosome fusion. LAMP-2B mutations underscore the myopathy and severe hypertrophic cardiomyopathy in Danon disease which results from deficits in autophagy (1, 4). Vasculopathy of coronary and cerebral arteries is a rare phenotype in Danon patients that is also associated with deficient autophagy processing of proteins and cellular organelles (5). LAMP2C serves as a receptor for DNA and RNA, facilitating their lysosomal degradation through DNA-autophagy and RNA-autophagy, respectively (1).

References

1. Rowland, T. J., Sweet, M. E., Mestroni, L., & Taylor, M. R. G. (2016). Danon disease - dysregulation of autophagy in a multisystem disorder with cardiomyopathy. Journal of Cell Science. https://doi.org/10.1242/jcs.184770

2. Alfaro, I. E., Albornoz, A., Molina, A., Moreno, J., Cordero, K., Criollo, A., & Budini, M. (2019). Chaperone mediated autophagy in the crosstalk of neurodegenerative diseases and metabolic disorders. Frontiers in Endocrinology. https://doi.org/10.3389/fendo.2018.00778

3. Schneider, J. L., & Cuervo, A. M. (2014). Autophagy and human disease: Emerging themes. Current Opinion in Genetics and Development. https://doi.org/10.1016/j.gde.2014.04.003

4. Chi, C., Leonard, A., Knight, W. E., Beussman, K. M., Zhao, Y., Cao, Y., Song, K. (2019). LAMP-2B regulates human cardiomyocyte function by mediating autophagosome lysosome fusion. Proceedings of the National Academy of Sciences of the United States of America. https://doi.org/10.1073/pnas.1808618116

5. Nguyen, H. T., Noguchi, S., Sugie, K., Matsuo, Y., Nguyen, C. T. H., Koito, H., Tsukaguchi, H. (2018). Small-Vessel Vasculopathy Due to Aberrant Autophagy in LAMP-2 Deficiency. Scientific Reports. https://doi.org/10.1038/s41598-018-21602-8

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Array NBP2-22217MFV500

• LAMP-2/CD107b Antibodies
• LAMP-2/CD107b ELISA Kits
• LAMP-2/CD107b Lysate
• LAMP-2/CD107b Proteins
• LAMP-2/CD107b Proteins and Enzymes