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  • Gene/Protein: PLEC PLEC1
  • Product Description: Immunotag™ PLEC Polyclonal Antibody
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Immunotag™ PLEC Polyclonal Antibody
Antibody Specification
Target Protein PLEC
Clonality Polyclonal
Storage/Stability -20°C/1 year
Application IHC-p
Recommended Dilution IHC-p 1:50-300
Concentration 1 mg/ml
Reactive Species Human,Rat,Mouse
Host Species Rabbit
Immunogen Synthesized peptide derived from human protein, at AA range: 240-320
Specificity PLEC Polyclonal Antibody detects endogenous levels of protein.
Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen
Form Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Gene Name PLEC PLEC1
Accession No. Q15149 Q9QXS1 P30427
Description plectin(PLEC) Homo sapiens Plectin is a prominent member of an important family of structurally and in part functionally related proteins, termed plakins or cytolinkers, that are capable of interlinking different elements of the cytoskeleton. Plakins, with their multi-domain structure and enormous size, not only play crucial roles in maintaining cell and tissue integrity and orchestrating dynamic changes in cytoarchitecture and cell shape, but also serve as scaffolding platforms for the assembly, positioning, and regulation of signaling complexes (reviewed in PMID: 9701547, 11854008, and 17499243). Plectin is expressed as several protein isoforms in a wide range of cell types and tissues from a single gene located on chromosome 8 in humans (PMID: 8633055, 8698233). Until 2010, this locus was named plectin 1 (symbol PLEC1 in human; Plec1 in mouse and rat) and the gene product had been referred to as "hemidesmosomal protein 1" or &qu
Protein Expression Epithelium,Hepatocyte,Ovarian carcinoma,Placenta,Skin,
Subcellular Localization cytoplasm,cytosol,cytoskeleton,plasma membrane,brush border,cell-cell adherens junction,focal adhesion,sarcoplasm,hemidesmosome,extracellular matrix,sarcolemma,costamere,intermediate filament cytoskeleton,extracellular exo
Protein Function disease:Defects in PLEC1 are the cause of epidermolysis bullosa simplex Ogna type (O-EBS) [MIM:131950]; also called epidermolysis bullosa simplex 1. O-EBS is a form of intraepidermal epidermolysis bullosa characterized by generalized skin bruising, skin fragility with non-scarring blistering and small hemorrhagic blisters on hands. At the ultrastructural level, it is differentiated from classical cases of K-EBS, WC-EBS and DM-EBS, by the occurrence of blisters originating in basal cells above hemidesmosomes, and abnormal hemidesmosome intracellular attachment plates.,disease:Defects in PLEC1 are the cause of epidermolysis bullosa simplex with muscular dystrophy (MD-EBS) [MIM:226670]. MD-EBS is an autosomal recessive disorder characterized by epidermal blister formation at the level of the hemidesmosome and associated with late-onset muscular dystrophy.,disease:Defects in PLEC1 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.,domain:The N-terminus interacts with actin, the C-terminus with vimentin, desmin, GFAP, cytokeratins, lamin B; whereas both the N-and the C-terminus can bind integrin beta-4.,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 cross-linking and stabilization of cytoskeletal intermediate filaments network, but also in the regulation of their dynamics.,online information:Plectin entry,PTM:Phosphorylated by CDC2; regulates dissociation from intermediate filaments during mitosis (By similarity). Phosphorylated upon DNA damage, probably by ATM or ATR. Isoform 4 is phosphorylated on 'Tyr-26' (By similarity). Isoform 4 is phosphorylated on 'Ser-21'.,similarity:Belongs to the plakin or cytolinker family.,similarity:Contains 1 actin-binding domain.,similarity:Contains 2 CH (calponin-homology) domains.,similarity:Contains 33 plectin repeats.,similarity:Contains 4 spectrin repeats.,subunit:Homodimer or homotetramer. Interacts with Nesprin-3.,tissue specificity:Widely expressed with highest levels in muscle, heart, placenta and spinal cord.,
Usage For Research Use Only! Not for diagnostic or therapeutic procedures.
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