Anti-TMPRSS3 Antibody Picoband®, PE Conjugate

Transmembrane protease, serine 3 is an enzyme that in humans is encoded by the TMPRSS3 gene. This gene encodes a protein that belongs to the serine protease family. The encoded protein contains a serine protease domain, a transmembrane domain, an LDL receptor-like domain, and a scavenger receptor cysteine-rich domain. Serine proteases are known to be involved in a variety of biological processes, whose malfunction often leads to human diseases and disorders. This gene was identified by its association with both congenital and childhood onset autosomal recessive deafness. This gene is expressed in fetal cochlea and many other tissues, and is thought to be involved in the development and maintenance of the inner ear or the contents of the perilymph and endolymph. This gene was also identified as a tumor-associated gene that is overexpressed in ovarian tumors. Alternatively spliced transcript variants have been described.

Mainly expressed in testis. Isoform 3 is expressed predominantly in adult testis, weakly in pancreas, embryonic testis and sperm, and at very low levels in other organs.

s the termination of nascent peptide synthesis (translation) in response to the termination codons UAA, UAG and UGA. Component of the transient SURF complex which recruits UPF1 to stalled ribosomes in the context of nonsense-mediated decay (NMD) of mRNAs containing premature stop codons. Required for SHFL-mediated translation termination which inhibits programmed ribosomal frameshifting (-1PRF) of mRNA from viruses and cellular genes.

1. Ben-Yosef, T., Wattenhofer, M., Riazuddin, S., Ahmed, Z. M., Scott, H. S., Kudoh, J., Shibuya, K., Antonarakis, S. E., Bonne-Tamir, B., Radhakrishna, U., Naz, S., Ahmed, Z., Riazuddin, S., Pandya, A., Nance, W. E., Wilcox, E. R., Friedman, T. B., Morell, R. J. Novel mutations of TMPRSS3 in four DFNB8/B10 families segregating congenital autosomal recessive deafness. (Letter) J. Med. Genet. 38: 396-400, 2001. 2. Fasquelle, L., Scott, H. S., Lenoir, M., Wang, J., Rebillard, G., Goboyard, S., Venteo, S., Francois, F., Mausset-Bonnefont, A.-L., Antonarakis, S. E., Neidhart, E., Chabbert, C., Puel, J.-L., Guipponi, M., Delprat, B. Tmprss3, a transmembrane serine protease deficient in human DFNB8/10 deafness, is critical for cochlear hair cell survival at the onset of hearing. J. Biol. Chem. 286: 17383-17397, 2011. 3. Guipponi, M., Vuagniaux, G., Wattenhofer, M., Shibuya, K., Vazquez, M., Dougherty, L., Scamuffa, N., Guida, E., Okui, M., Rossier, C., Hancock, M., Buchet, K., Reymond, A., Hummler, E., Marzella, P. L., Kudoh, J., Shimizu, N., Scott, H. S., Antonarakis, S. E., Rossier, B. C. The transmembrane serine protease (TMPRSS3) mutated in deafness DFNB8/10 activates the epithelial sodium channel (ENaC) in vitro. Hum. Molec. Genet. 11: 2829-2836, 2002.

Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Western blot, 0.25-0.5μg/ml, Human, Mouse, Rat<br> Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml, Human, Mouse, Rat<br> Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human<br> ELISA, 0.1-0.5μg/ml, -<br>