ACTN2 Monoclonal antibody, PBS Only

Alpha actinin 2 (ACTN2) belongs to the alpha-actinin family and is expressed in both skeletal and cardiac muscles and functions to anchor myofibrillar actin thin filaments and titin to Z-discs (PMID: 30701273). ACTN2 is an actin-binding protein with multiple roles in different cell types. In nonmuscle cells, the cytoskeletal isoform is found along microfilament bundles and adherens-type junctions, where it is involved in binding actin to the membrane. In contrast, skeletal, cardiac, and smooth muscle isoforms are localized to the Z disc and analogous dense bodies, where they help anchor the myofibrillar actin filaments. Mutations in ACTN2 are associated with hypertrophic cardiomyopathy, as well as dilated cardiomyopathy and endocardial fibroelastosis (PMID: 20022194, 14567970).