Anti-Desmin Antibody [SI18-00]

ET1606-30

Anti-Desmin Antibody [SI18-00]

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.

496.5 €

A2001-100

Anti-Desmin Antibody

758.4 €

ER40113

Anti-Desmin Antibody

Desmin is one of the earliest protein markers for muscle tissue in embryogenesis as it is detected in the somites. Although it is present early in the development of muscle cells, it is only expressed at low levels, and increases as the cell nears terminal differentiation. Desmin is also important in muscle cell architecture and structure since it connects many components of the cytoplasm. Finally, desmin may be important in mitochondria function. Desmin-related myopathy (DRM or Desminopathy) is a subgroup of the myofibrillar myopathy diseases and is the result of a mutation in the gene that codes for desmin which prevents it from forming protein filaments, instead forming aggregates of desmin and other proteins throughout the cell. Recently, mutations were identified in patients suffered by an arrhythmogenic right ventricular cardiomyopathy (ARVC).

433.5 €

MBS175046-01mg

Anti-Desmin antibody (monoclonal)

758.75 €

MBS175046-5x01mg

Anti-Desmin antibody (monoclonal)

2569.25 €

MBS9510670-005mL

Anti-Desmin Antibody [ABT-DES]

427.25 €