Anti-Desmin

0.5mg/ml if reconstituted with 0.2ml sterile DI water. DES, also called Desmin, is a protein that in humans is encoded by the DES gene, and this gene is mapped to 2q35. DES is a member of the intermediate filament (IF) protein family. It is one of the earliest myogenic markers, both in heart and somites, and is expressed in satellite stem cells and replicating myoblasts. It is very important in muscle cell architecture and structure since it connects many components of the cytoplasm. It may be also play an important role in mitochondria function. Additionally, DES provides attachments between the terminal Z disc and membrane-associated proteins to form a force-transmitting system that parallels the thin filaments at myotendinous junctions. Protein function: Muscle-specific type III intermediate filament essential for proper muscular structure and function. Plays a crucial role in maintaining the structure of sarcomeres, inter-connecting the Z-disks and forming the myofibrils, linking them not only to the sarcolemmal cytoskeleton, but also to the nucleus and mitochondria, thus providing strength for the muscle fiber during activity (PubMed:25358400). In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z- line structures (PubMed:24200904, PubMed:25394388, PubMed:26724190). May act as a sarcomeric microtubule-anchoring protein: specifically associates with detyrosinated tubulin-alpha chains, leading to buckled microtubules and mechanical resistance to contraction. Required for nuclear membrane integrity, via anchoring at the cell tip and nuclear envelope, resulting in maintenance of microtubule-derived intracellular mechanical forces. Contributes to the transcriptional regulation of the NKX2-5 gene in cardiac progenitor cells during a short period of cardiomyogenesis and in cardiac side population stem cells in the adult. Plays a role in maintaining an optimal conformation of nebulette (NEB) on heart muscle sarcomeres to bind and recruit cardiac alpha-actin. [The UniProt Consortium]