Chen Li, Derek T Warren, Can Zhou, Shanelle De Silva, Darren G S Wilson, Mitla Garcia-Maya, Mathew A Wheeler, Peter Meinke, Greta Sawyer, Elisabeth Ehler, Manfred Wehnert, Li Rao, Qiuping Zhang, Catherine M Shanahan
Nesprins comprise a family of multi-isomeric scaffolding proteins, forming the linker of nucleoskeleton-and-cytoskeleton complex with lamin A/C, emerin and SUN1/2 at the nuclear envelope. Mutations in nesprin-1/-2 are associated with Emery-Dreifuss muscular dystrophy (EDMD) with conduction defects and dilated cardiomyopathy (DCM). We have previously observed sarcomeric staining of nesprin-1/-2 in cardiac and skeletal muscle, but nesprin function in this compartment remains unknown. In this study we show that specific nesprin-2 isoforms are highly expressed in cardiac muscle and localise to the Z-disc and I band of the sarcomere...
April 1, 2024: Journal of Biological Chemistry