Anti-iASPP Antibody, clone LX128.5 clone LX128.5, from mouse

RelA-associated inhibitor (UniProt Q8WUF5; also known as Inhibitor of apoptosis-stimulating protein of p53, Inhibitor of ASPP protein, NF-kB-interacting protein 1, PPP1R13B-like protein, Protein iASPP) is encoded by the PPP1R13L (also known as IASPP, NKIP1, PPP1R13BL, RAI) gene (Gene ID 10848) in human. Inhibitor of apoptosis-stimulating protein of p53 (iASPP) belongs to the ASPP family of proteins (ASPP1, ASPP2, and iASPP) characterized by their ankyrin repeats (a.a. 659-691 & 692-724 in iASPP), SH3 domain (a.a. 758-820 in iASPP), and proline-rich (a.a. 54-602 in iASPP) sequences. iASPP is expressed at intercalated discs in post-mitotic cardiomyocytes, where it interacts with desmoplakin and desmin in cardiomyocytes to help maintain the integrity of desmosomes and intermediate filament networks. Desmosomes are anchoring junctions found in cells that endure physical stress such as cardiac myocytes or skin keratinocytes. Mutations of genes coding desmosome components, including IASPP/PPP1R13L, are found in human patients suffering from cardiomyopathy and in animal models of cardiomyopathy. iASPP deficiency caused by spontaneous mutation has been reported to be linked to a lethal autosomal recessive cardiomyopathy in Poll Hereford calves and Wa3 mice. iASSP-deficient mice with exon 8 deletion display features of arrhythmogenic right ventricular cardiomyopathy (ARVC) and die of sudden cardiac death.