DSP

Desmoplakin (DSP) is a major structural protein of desmosomes, highly expressed in cardiac muscle and stratified epithelia. It anchors intermediate filaments to cell–cell junctions, maintaining tissue integrity and mechanical stability in the heart and skin.
Mutations in DSP cause inherited disorders affecting the heart and skin, including arrhythmogenic right ventricular cardiomyopathy and epidermal fragility syndromes. Current management is largely supportive. Gene therapies and small-molecule stabilizers are under preclinical development to restore junctional function and reduce disease progression.