P17661

Cytoskeletal intermediate filaments (IFs) constitute a diverse group of proteins that are expressed in a highly tissue-specific manner. IFs are constructed from two-chain α-helical coiled-coil molecules arranged on an imperfect helical lattice, and have been widely used as markers for distinguishing individual cell types within a tissue and identifying the origins of metastatic tumors. Vimentin is an IF general marker of cells originating in the mesenchyme. Vimentin and Desmin, a related class III IF, are both expressed during skeletal muscle development. Desmin, a 469 amino acid protein found near the Z line in sarcomeres, is expressed more frequently in adult differentiated state tissues. Desmin makes up attachments between the terminal Z-disc and membrane-associated proteins to form a force-transmitting system. Mutations in the gene encoding for Desmin are associated with adult-onset skeletal myopathy, sporadic disease and mild cardiac involvement.