Rabbit anti-Human PLEC Polyclonal Antibody

Uniprot Id

Q15149

Target Species

Human

Target Name

PLEC

Target Full Name

Plectin

Target Function

Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofiber integrity.

Target Involvement

Epidermolysis bullosa simplex with pyloric atresia (EBS-PA); Epidermolysis bullosa simplex, with muscular dystrophy (MD-EBS); Epidermolysis bullosa simplex, Ogna type (O-EBS); Limb-girdle muscular dystrophy 2Q (LGMD2Q); Epidermolysis bullosa simplex with nail dystrophy (EBSND)

Target Subcellular Location

Cytoplasm, cytoskeleton. Cell junction, hemidesmosome.

Target Protein Families

Plakin or cytolinker family

Target Tissue Specificity

Widely expressed with highest levels in muscle, heart, placenta and spinal cord.

Target Synonyms

EBS1; EBSO; HD1; Hemidesmosomal protein 1; PCN; pleC; PLEC_HUMAN; PLEC1; PLEC1b; Plectin 1; Plectin 1 intermediate filament binding protein 500kDa; Plectin 6; Plectin; Plectin-1; PLTN

Target 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 .