Anti-SCN1B Antibody Picoband® Fluoro488 Conjugated

Voltage-gated sodium (Na+) channels are essential for the generation and propagation of action potentials in striated muscle and neuronal tissues. The complete coding region of Sodium channel, voltage-gated, beta 1 (SCN1B) is found in approximately 9.0 kb of genomic DNA and consists of five exons (72 to 749 bp) and four introns (90 bp to 5.5 kb) . It is mapped to 19q13.1 and can act as a candidate gene for hereditary disorders affecting membrane excitability. Sodium channel beta1 subunits play important roles in the regulation of sodium channel density and localization and are involved in axo-glial communication at nodes of Ranvier.

Ubiquitin carboxyl-terminal hydrolase 15; Deubiquitinating enzyme 15; Ubiquitin thioesterase 15; Ubiquitin-specific-processing protease 15; Unph-2; Unph4; USP15; KIAA0529

Hydrolase that removes conjugated ubiquitin from target proteins and regulates various pathways such as the TGF-beta receptor signaling, NF-kappa-B and RNF41/NRDP1-PRKN pathways. Acts as a key regulator of TGF-beta receptor signaling pathway, but the precise mechanism is still unclear: according to a report, acts by promoting deubiquitination of monoubiquitinated R-SMADs (SMAD1, SMAD2 and/or SMAD3), thereby alleviating inhibition of R-SMADs and promoting activation of TGF-beta target genes. According to another reports, regulates the TGF-beta receptor signaling pathway by mediating deubiquitination and stabilization of TGFBR1, leading to an enhanced TGF-beta signal. Able to mediate deubiquitination of monoubiquitinated substrates as well as 'Lys-48'-linked polyubiquitin chains, protecting them against proteasomal degradation. May also regulate gene expression and/or DNA repair through the deubiquitination of histone H2B. Acts as an inhibitor of mitophagy by counteracting the action of parkin (PRKN) : hydrolyzes cleavage of 'Lys-48'- and 'Lys-63'-linked polyubiquitin chains attached by parkin on target proteins such as MFN2, thereby reducing parkin's ability to drive mitophagy. Acts as an associated component of COP9 signalosome complex (CSN) and regulates different pathways via this association: regulates NF-kappa-B by mediating deubiquitination of NFKBIA and deubiquitinates substrates bound to VCP. Involved in endosome organization by mediating deubiquitination of SQSTM1: ubiquitinated SQSTM1 forms a molecular bridge that restrains cognate vesicles in the perinuclear region and its deubiquitination releases target vesicles for fast transport into the cell periphery. (Microbial infection) Protects APC and human papillomavirus type 16 protein E6 against degradation via the ubiquitin proteasome pathway.

1. Makita, N.; Sloan-Brown, K.; Weghuis, D. O.; Ropers, H. H.; George, A. L., Jr. : Genomic organization and chromosomal assignment of the human voltage-gated Na (+) channel beta-1 subunit gene (SCN1B) . Genomics 23: 628-634, 1994. 2. Chen, C.; Westenbroek, R. E.; Xu, X.; Edwards, C. A.; Sorenson, D. R.; Chen, Y.; McEwen, D. P.; O'Malley, H. A.; Bharucha, V.; Meadows, L. S.; Knudsen, G. A.; Vilaythong, A.; Noebels, J. L.; Saunders, T. L.; Scheuer, T.; Shrager, P.; Catterall, W. A.; Isom, L. L. : Mice lacking sodium channel beta-1 subunits display defects in neuronal excitability, sodium channel expression, and nodal architecture. J. Neurosci. 24: 4030-4042, 2004.