PLEC Antibody Blocking Peptide (合成多肽) | Bioss

PLEC

Plectin

Homodimer or homotetramer. Interacts (via actin-binding domain) with Nesprin-3. Interacts (via CH 1 domain) with VIM (via rod region). Interacts with FER (By similarity). Interacts with COL17A1 (via N-terminus). Interacts (via N-terminus) with DST isoform 1 (via N-terminus).

Cytoplasm, cytoskeleton. Cell junction, hemidesmosome.

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

Phosphorylated by CDK1; regulates dissociation from intermediate filaments during mitosis. Phosphorylated upon DNA damage, probably by ATM or ATR.

Defects in PLEC are the cause of epidermolysis bullosa simplex with pyloric atresia (EBS-PA) [MIM:612138]. EBS-PA is an autosomal recessive genodermatosis characterized by severe skin blistering at birth and congenital pyloric atresia. Death usually occurs in infancy. This disorder is allelic to MD-EBS.

Belongs to the plakin or cytolinker family.
Contains 1 actin-binding domain.
Contains 2 CH (calponin-homology) domains.
Contains 33 plectin repeats.
Contains 4 spectrin repeats.

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 myofibers integrity.