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Cytoskeletal linker protein. Acts as an integrator of intermediate filaments, actin and microtubule cytoskeleton networks. Required for anchoring either intermediate filaments to the actin cytoskeleton in neural and muscle cells or keratin-containing intermediate filaments to hemidesmosomes in epithelial cells. The proteins may self-aggregate to form filaments or a two-dimensional mesh.Isoform 3: plays a structural role in the assembly of hemidesmosomes of epithelial cells; anchors keratin-containing intermediate filaments to the inner plaque of hemidesmosomes. Required for the regulation of keratinocyte polarity and motility; mediates integrin ITGB4 regulation of RAC1 activity.Isoform 6: required for bundling actin filaments around the nucleus.
Gene Name: | dystonin |
Synonyms: | DST, BP230, BP240, BPA, BPAG1, Bullous pemphigoid antigen, CATX-15, CATX15, D6S1101, Dystonia musculorum protein, DMH, Dystonin, Hemidesmosomal plaque protein, HSAN6, KIAA0728, MACF2, KIAA0465, KIAA1470, Trabeculin-beta, Bullous pemphigoid antigen 1, DT |
Target Sequences: | NM_183380 NP_899236.1 Q03001 |
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