Anti-SRP54 Antibody Picoband® Fluoro647 Conjugated

The signal recognition particle (SRP) is a ribonucleoprotein complex that mediates the targeting of proteins to the endoplasmic reticulum (ER) . The complex consists of a 7S (or 7SL) RNA and 6 different proteins, and signal recognition particle 54 (SRP54) is one of them. SRP54 binds to the signal sequence of presecretory protein as they emerge from the translating ribosomes, and then transfers them to translocating chain-associating membrane protein (TRAM) .

Ubiquitin carboxyl-terminal hydrolase 25; Deubiquitinating enzyme 25; USP on chromosome 21; Ubiquitin thioesterase 25; Ubiquitin-specific-processing protease 25; USP25; USP21

Isoform USP25a is found in most adult and fetal tissues; expression is moderately high in testis, pancreas, kidney, skeletal muscle, liver, lung, placenta, brain, heart, but very low in peripheral blood, colon, small intestine, ovary, prostate, thymus and spleen. Isoform USP25b is found in all tissues except heart and skeletal muscle. Isoform USP25m is heart and skeletal muscle specific.

Deubiquitinating enzyme that hydrolyzes ubiquitin moieties conjugated to substrates and thus, functions to process newly synthesized Ubiquitin, to recycle ubiquitin molecules or to edit polyubiquitin chains and prevents proteasomal degradation of substrates. Hydrolyzes both 'Lys-48'- and 'Lys-63'-linked tetraubiquitin chains. The muscle-specific isoform (USP25m) may have a role in the regulation of muscular differentiation and function.

1. Bellanne-Chantelot, C., Schmaltz-Panneau, B., Marty, C., Fenneteau, O., Callebaut, I., Clauin, S., Docet, A., Damaj, G.-L., Leblanc, T., Pellier, I., Stoven, C., Souquere, S., and 26 others. Mutations in the SRP54 gene cause severe congenital neutropenia as well as Shwachman-Diamond-like syndrome. Blood 132: 1318-1331, 2018. 2. Carapito, R., Konantz, M., Paillard, C., Miao, Z., Pichot, A., Leduc, M. S., Yang, Y., Bergstrom, K. L., Mahoney, D. H., Shardy, D. L., Alsaleh, G., Naegely, L., and 38 others. Mutations in signal recognition particle SRP54 cause syndromic neutropenia with Shwachman-Diamond-like features. J. Clin. Invest. 127: 4090-4103, 2017. 3. Gowda, K., Chittenden, K., Zwieb, C. Binding site of the M-domain of human protein SRP54 determined by systematic site-ed mutagenesis of signal recognition particle RNA. Nucleic Acids Res. 25: 388-394, 1997.