Dystonin

**Definition:** A plakin characterized by repeat sequences homologous to SPECTRIN and PLECTIN and C-terminal EF HAND MOTIFS. It functions as an integrator of INTERMEDIATE FILAMENTS, ACTIN and MICROTUBULES in cytoskeleton networks. It is required for anchoring intermediate filaments to the actin cytoskeleton in neural and muscle cells as well as anchoring KERATIN-containing intermediate filaments to HEMIDESMOSOMES in EPITHELIAL CELLS.

**Tree numbers:** - D12.776.220.790.625

**Synonyms:** - Hemidesmosomal Plaque Protein - 230 kDa Bullous Pemphigoid Antigen - 230-240 kDa Bullous Pemphigoid Antigen - Bullous Pemphigoid Antigen 1