Anti-Scavenging Receptor SRB2/SCARB2 Antibody Picoband® Fluoro488 Conjugated

Lysosomal integral membrane protein 2 (LIMP-2) is a protein that in humans is encoded by the SCARB2 gene. The protein encoded by this gene is a type III glycoprotein that is located primarily in limiting membranes of lysosomes and endosomes. Earlier studies in mice and rat suggested that this protein may participate in membrane transportation and the reorganization of endosomal/lysosomal compartment. The protein deficiency in mice was reported to impair cell membrane transport processes and cause pelvic junction obstruction, deafness, and peripheral neuropathy. Further studies in human showed that this protein is a ubiquitously expressed protein and that it is involved in the pathogenesis of HFMD (hand, foot, and mouth disease) caused by enterovirus-71 and possibly by coxsackievirus A16. Mutations in this gene caused an autosomal recessive progressive myoclonic epilepsy-4 (EPM4), also known as action myoclonus-renal failure syndrome (AMRF) . Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Protease that deconjugates SUMO1, SUMO2 and SUMO3 from targeted proteins. Processes preferentially poly-SUMO2 and poly-SUMO3 chains, but does not efficiently process SUMO1, SUMO2 and SUMO3 precursors. Deconjugates SUMO1 from RXRA, leading to transcriptional activation. Involved in chromosome alignment and spindle assembly, by regulating the kinetochore CENPH-CENPI-CENPK complex. Desumoylates PML and CENPI, protecting them from degradation by the ubiquitin ligase RNF4, which targets polysumoylated proteins for proteasomal degradation. Desumoylates also RPA1, thus preventing recruitment of RAD51 to the DNA damage foci to initiate DNA repair through homologous recombination.

1. Badhwar, A., Berkovic, S. F., Dowling, J. P., Gonzales, M., Narayanan, S., Brodtmann, A., Berzen, L., Caviness, J., Trenkwalder, C., Winkelmann, J., Rivest, J., Lambert, M., Hernandez-Cossio, O., Carpenter, S., Andermann, F., Andermann, E. Action myoclonus-renal failure syndrome: characterization of a unique cerebro-renal disorder. Brain 127: 2173-2182, 2004. 2. Balreira, A., Gaspar, P., Caiola, D., Chaves, J., Beirao, I., Lopes Lima, J., Azevedo, J. E., Sa Miranda, M. C. A nonsense mutation in the LIMP-2 gene associated with progressive myoclonic epilepsy and nephrotic syndrome. Hum. Molec. Genet. 17: 2238-2243, 2008. 3. Berkovic, S. F., Dibbens, L. M., Oshlack, A., Silver, J. D., Katerelos, M., Vears, D. F., Lullmann-Rauch, R., Blanz, J., Zhang, K. W., Stankovich, J., Kalnins, R. M., Dowling, J. P., and 14 others. Array-based gene discovery with three unrelated subjects shows SCARB2/LIMP-2 deficiency causes myoclonus epilepsy and glomerulosclerosis. Am. J. Hum. Genet. 82: 673-684, 2008.