| Function | 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. Regulates the organization and stability of the microtubule network of sensory neurons to allow axonal transport. Mediates docking of the dynein/dynactin motor complex to vesicle cargos for retrograde axonal transport through its interaction with TMEM108 and DCTN1. 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. Isoform 7: Regulates the organization and stability of the microtubule network of sensory neurons to allow axonal transport. |
| Protein Name | Dystonin230 Kda Bullous Pemphigoid Antigen230/240 Kda Bullous Pemphigoid AntigenBullous Pemphigoid Antigen 1BpaBullous Pemphigoid AntigenDystonia Musculorum ProteinHemidesmosomal Plaque Protein |
| Database Links | Reactome: R-HSA-2022090 Q03001-3Reactome: R-HSA-446107Reactome: R-HSA-9013420Reactome: R-HSA-9013424Reactome: R-HSA-9696264Reactome: R-HSA-9696270Reactome: R-HSA-9696273 |
| Cellular Localisation | CytoplasmCytoskeletonStress FiberCell ProjectionAxonAssociates With Intermediate FilamentsActin And Microtubule CytoskeletonsLocalizes To Actin Stress Fibers And To Actin-Rich Ruffling At The Cortex Of CellsAssociated At The Growing Distal Tip Of MicrotubulesIsoform 1: CytoplasmMyofibrilSarcomereZ LineH ZoneLocalizes To Microtubules And Actin Microfilaments Throughout The Cytoplasm And At Focal Contact Attachments At The Plasma MembraneIsoform 2: CytoplasmColocalizes Both Cortical And Cytoplasmic Actin FilamentsIsoform 3: CytoplasmCell JunctionHemidesmosomeLocalizes To Actin And Intermediate Filaments CytoskeletonsColocalizes With The Epidermal Krt5-Krt14 Intermediate Filaments Network Of KeratinsColocalizes With Itgb4 At The Leading Edge Of Migrating KeratinocytesIsoform 6: NucleusNucleus EnvelopeMembraneSingle-Pass Membrane ProteinEndoplasmic Reticulum MembraneLocalizes To Central Actin Stress Fibers Around The Nucleus And Is Excluded Form Focal Contact Sites In Myoblast CellsTranslocates To The NucleusAssociates With Actin Cytoskeleton In Sensory NeuronsIsoform 7: CytoplasmAssociates With Axonal Microtubules And Intermediate FilamentsBut Not With Actin CytoskeletonIn Sensory NeuronsIsoform 8: CytoplasmCell CortexCell MembraneLipid-Anchor |
| Alternative Protein Names | Dystonin protein230 Kda Bullous Pemphigoid Antigen protein230/240 Kda Bullous Pemphigoid Antigen proteinBullous Pemphigoid Antigen 1 proteinBpa proteinBullous Pemphigoid Antigen proteinDystonia Musculorum Protein proteinHemidesmosomal Plaque Protein proteinDST proteinBP230 proteinBP240 proteinBPAG1 proteinDMH proteinDT proteinKIAA0728 protein |
Information sourced from Uniprot.org
