Desmin Rabbit polyclonal Antibody IgG
Fig1: Western blot analysis of Desmin on different cell lysates using anti-Desmin antibody at 1/2000 dilution.
Lane 1: Human skeletal muscle tissue
Lane 2: Mouse heart tissue
Lane 3: Human heart tissue
Fig2: ICC staining of Desmin in HepG2 cells (green). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.
Fig3: ICC staining of Desmin in NIH-3T3 cells (green). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.
Host Species; Species ReactivityRabbit; Human, Mouse, Zebrafish
Application SummaryWB, ICC, IHC
Purification; FormulationPeptide affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
BackgroundDesmin 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).(ER40113)