Desmin antibody - HS-511 008

Desmin is the most important muscular intermediate filament (IF) and is expressed in cardiac, skeletal and smooth muscles. Desmin IFs envelop myofibrils at the level of Z-disks and extend from the nuclear envelope to the sarcolemma and various organelles such as nuclei, mitochondria, and the sarcoplasmic reticulum (1). In this way, desmin IFs contribute to structural and mechanical support, cellular integrity, force transmission, and mitochondrial homeostasis (1). Desmin knockout-mice develop normally and are fertile. However, after birth mice develop cardiovascular lesions and skeletal myopathy (2). Studies in these mice suggest that desmin is not required for myogenic commitment, differentiation or fusion, but is essential for maintaining the tensile strength and integrity of muscle fibers (3). In humans, missense mutations in the desmin gene (DES) also cause a distinct “desmin myopathy”, which is often associated with cardiomyopathy (4).