Dystonin

3GJO667n/aENSG00000151914n/aQ03001n/aNM_020388NM_183380n/aNP_001138241NP_001138242NP_001714NP_056363NP_899236n/aDystonin (DST), also known as Bullous pemphigoid antigen 1,(BPAG1), isoforms 1/2/3/4/5/8, is a protein that in humans is encoded by the DST gene.2iak: Crystal Structure of a protease resistant fragment of the plakin domain of Bullous Pemphigoid Antigen1 (BPAG1) Dystonin (DST), also known as Bullous pemphigoid antigen 1,(BPAG1), isoforms 1/2/3/4/5/8, is a protein that in humans is encoded by the DST gene. This gene encodes a member of the plakin protein family of adhesion junction plaque proteins. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene, but the full-length nature of some variants has not been defined. It has been known that some isoforms are expressed in neural and muscle tissue, anchoring neural intermediate filaments to the actin cytoskeleton, and some isoforms are expressed in epithelial tissue, anchoring keratin-containing intermediate filaments to hemidesmosomes. Consistent with the expression, mice defective for this gene show skin blistering and neurodegeneration. Dystonin has been shown to interact with Collagen, type XVII, alpha 1, DCTN1 and Erbin.