Rabbit polyclonal antibody to Actinin~α3 (E25)

The spectrin gene family encodes a diverse group of cytoskeletal proteins that include spectrins, dystrophins and α~actinins. There are four tissue~specific α~actinins, namely α~actinin~1, α~actinin~2, α~actinin~3 and α~actinin~4, which are localized to muscle and non~muscle cells, including skeletal, cardiac and smooth muscle cells, as well as within the cytoskeleton. Each α~actinin protein contains one Actin~binding domain, two calponin~homology domains, two EF~hand domains and four spectrin repeats, through which they function as bundling proteins that can cross~link F~Actin, thus anchoring Actin to a variety of intracellular structures. Defects in the gene encoding α~actinin~4 are the cause of focal segmental glomerulosclerosis 1 (FSGS1), a common renal lesion characterized by decreasing kidney function and, ultimately, renal failure.