Synonyms: HD1; PCN; EBS1; EBSO; PLTN; EBS5A; EBS5B; EBS5C; EBS5D; EBSMD; EBSND; EBSOG; EBSPA; PLEC1; LGMD2Q; PLEC1b; LGMDR17; Plectin Background: Plectin is a large, widely expressed protein that crosslinks the intermediate filament and actin cytoskeleton, mechanically stabilizing cells and tissues. Plectin also plays a role in the regulation of actin dynamics and acts as a scaffold for signaling molecules. Plectin is important in the stabilization of hemidesmosomes, crosslinking them to the intermediate filament network. Research studies have shown that mutations in plectin and other genes coding for hemidesmosomal proteins can cause epidermolysis bullosa, a condition manifested by fragile skin and frequent blistering . Plectin modulates signals to PKC through binding and sequestration of RACK1, the receptor for activated C kinase 1 . Plectin is also involved in the regulation of cytokeratin architecture and cell stress response , signaling through the chemokine receptor CXCR4 and regulation of AMP-activated protein kinase (AMPK) activity and signaling in mouse myotubes .