Anti-Desmoplakin antibody [EPR4383(2)]

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Desmoplakin is a protein that plays an integral role in linking intermediate filaments to desmosomal plaques. This protein is also known by its alternate name "DSP" and has a molecular mass of approximately 332 kDa. Desmoplakin is expressed in tissues where mechanical strength is necessary such as the heart and skin. Its expression ensures structural integrity and resilience in areas that experience substantial mechanical stress.

Biological function summary

Desmoplakin acts to maintain cell-cell adhesion through its involvement with desmosomes and is an essential component of the desmosomal complex. This complex is important for the architectural stability of epithelial and cardiac tissues. Desmoplakin interacts directly with other desmosomal proteins like plakoglobin and plakophilin forming a bridge between the cell membrane and the cytoskeleton.

Pathways

Desmoplakin is an important player in the maintenance and function of the cytoskeletal network particularly within the intermediate filament organization. In addition desmoplakin participates in cellular adhesion signaling pathways. Its interaction with proteins such as plakoglobin mediates the organization of desmosomes influencing cellular signaling and communication processes critical for tissue morphogenesis and repair.

Associated diseases and disorders

Mutations or malfunctions of desmoplakin are linked to conditions like arrhythmogenic right ventricular cardiomyopathy (ARVC) and skin disorders such as striate palmoplantar keratoderma. In these diseases desmoplakin's ability to maintain structural integrity is compromised leading to the disruption of tissue architecture. In ARVC for example the faulty interaction between desmoplakin and plakoglobin may cause abnormal heart muscle function contributing to the pathology of the disease.