Rabbit Plectin Polyclonal Antibody | anti-Plectin antibody

Rabbit anti Plectin Polyclonal antibody

Cat. Num. AAA462240

• Gene Names: PLEC; HD1; PCN; EBS1; EBSO; PLTN; PLEC1; LGMD2Q; PLEC1b
• Reactivity: Human, Rat, Mouse
• Applications: ELISA, Western Blot
• Purity: The Rabbit IgG is purified by Epitope Affinity Purification.
• Synonyms: Plectin; Polyclonal Antibody; Rabbit anti Plectin Polyclonal antibody; Rabbit anti-Plectin; PLTN; PLEC1; intermediate filament binding protein 500kDa."; anti-Plectin antibody

• Host: Rabbit
• Reactivity: Human, Rat, Mouse
• Clonality: Polyclonal
• Specificity: This antibody recognizes ~500 kDa of Plectin protein. It reacts with human, mouse and rat. The other species are not tested.
• Purity/Purification: The Rabbit IgG is purified by Epitope Affinity Purification.
• Form/Format: This affinity purified antibody is supplied in sterile phosphate-buffered saline (pH7.2) containing antibody stabilizer
• Concentration: Size: 100 ug/200 ul (varies by lot)

• Applicable Applications for anti-Plectin antibody: ELISA, Western Blot
• Application Notes: Western Blot: 0.1-1 ug/ml; ELISA: 0.01-0.1 ug/ml; Immunoprecipitation: 2-5 ug/ml; Immunohistochemistry: 0.5-2 ug/ml
• Antigen Preparation: A synthetic peptide corresponding to the C-term portion of human Plectin. This sequence is identical to rat, mouse and human origins.
• Positive Control: Brain tissue
• Cellular Location: Cytoplasmic
• Preparation and Storage: The antibodies are stable for 12 months from date of receipt when stored at -20 degree C to -70 degree C. The antibodies can be stored at 2 degree C-8 degree C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.

Testing Data

Related Product Information for anti-Plectin antibody

Plectin is a cytoskeleton linker protein expressed in a variety of tissues including skin, muscle, and nerves. Plectin can bind to several molecules (intermediate filaments) that make up the cytoskeleton. Mutations in its gene are associated with epidermolysis bullosa simplex with late-onset muscular dystrophy. In certain skin cells, this protein is an essential part of hemidesmosomes, which attach the network of intermediate filaments to the cell membrane. It is also a component of desmosomes, which form junctions between neighboring cells. As part of these structures, plectin plays a critical role in anchoring the outer layer of the skin (the epidermis) to underlying layers. Mutations in the PLEC1 gene also cause at least two other forms of epidermolysis bullosa

References

Johann W. Bauer et al. A Compound Heterozygous One Amino-Acid Insertion/Nonsense Mutation in the Plectin Gene Causes Epidermolysis Bullosa Simplex with Plectin Deficiency. Am J Pathol. 2001 February; 158 (2): 617-625.

NCBI and Uniprot Product Information

• NCBI GI #: 1296662
• NCBI GeneID: 5339
• UniProt Accession #: Q15149; Q15148; Q16640; Q6S376; Q6S377; Q6S378; Q6S379; Q6S380; Q6S381; Q6S382; Q6S383; Q8WXV5; Q96IE3
• Molecular Weight: ~500 kDa
• NCBI Official Full Name: plectin
• NCBI Official Synonym Full Names: plectin
• NCBI Official Symbol: PLEC
• NCBI Official Synonym Symbols: HD1; PCN; EBS1; EBSO; PLTN; PLEC1; LGMD2Q; PLEC1b
• NCBI Protein Information: plectin; plectin-1; OTTHUMP00000229282; OTTHUMP00000229283; OTTHUMP00000229284; OTTHUMP00000229285; OTTHUMP00000229286; OTTHUMP00000229287; OTTHUMP00000229626; OTTHUMP00000229627; OTTHUMP00000229628; hemidesmosomal protein 1; plectin 1, intermediate filament binding protein 500kDa
• UniProt Protein Name: Plectin
• Protein Family: Plectin
• UniProt Gene Name: PLEC
• UniProt Synonym Gene Names: PLEC1
• UniProt Entry Name: PLEC_HUMAN

NCBI Description

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 (for reviews see 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 "plectin 1, intermediate filament binding 500kDa". These names have been superseded by plectin. The plectin gene locus in mouse on chromosome 15 has been analyzed in detail (PMID: 10556294, 14559777), revealing a genomic exon-intron organization with well over 40 exons spanning over 62 kb and an unusual 5' transcript complexity of plectin isoforms. Eleven exons (1-1j) have been identified that alternatively splice directly into a common exon 2 which is the first exon to encode plectin's highly conserved actin binding domain (ABD). Three additional exons (-1, 0a, and 0) splice into an alternative first coding exon (1c), and two additional exons (2alpha and 3alpha) are optionally spliced within the exons encoding the acting binding domain (exons 2-8). Analysis of the human locus has identified eight of the eleven alternative 5' exons found in mouse and rat (PMID: 14672974); exons 1i, 1j and 1h have not been confirmed in human. Furthermore, isoforms lacking the central rod domain encoded by exon 31 have been detected in mouse (PMID:10556294), rat (PMID: 9177781), and human (PMID: 11441066, 10780662, 20052759). It has been shown that the short alternative amino-terminal sequences encoded by the different first exons direct the targeting of the various isoforms to distinct subcellular locations (PMID: 14559777). As the expression of specific plectin isoforms was found to be dependent on cell type (tissue) and stage of development (PMID: 10556294, 12542521, 17389230) it appears that each cell type (tissue) contains a unique set (proportion and composition) of plectin isoforms, as if custom-made for specific requirements of the particular cells. Concordantly, individual isoforms were found to carry out distinct and specific functions (PMID: 14559777, 12542521, 18541706). In 1996, a number of groups reported that patients suffering from epidermolysis bullosa simplex with muscular dystrophy (EBS-MD) lacked plectin expression in skin and muscle tissues due to defects in the plectin gene (PMID: 8698233, 8941634, 8636409, 8894687, 8696340). Two other subtypes of plectin-related EBS have been described: EBS-pyloric atresia (PA) and EBS-Ogna. For reviews of plectin-related diseases see PMID: 15810881, 19945614. Mutations in the plectin gene related to human diseases should be named based on the position in NM_000445 (variant 1, isoform 1c), unless the mutation is located within one of the other alternative first exons, in which case the position in the respective Reference Sequence should be used.

Uniprot Description

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 maintainance of myofibers integrity. Ref.6 Ref.26

Subunit structure: Homodimer or homotetramer. Interacts (via actin-binding domain) with Nesprin-3. Interacts (via CH 1 domain) with VIM (via rod region)

By similarity. Interacts with COL17A1 (via N-terminus). Interacts (via N-terminus) with DST isoform 1 (via N-terminus). Ref.6 Ref.27

Subcellular location: Cytoplasm › cytoskeleton. Cell junction › hemidesmosome Ref.6.

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

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.

Post-translational modification: Phosphorylated by CDK1; regulates dissociation from intermediate filaments during mitosis

By similarity. Phosphorylated upon DNA damage, probably by ATM or ATR. Ref.5 Ref.7 Ref.8 Ref.9 Ref.10 Ref.11 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24

Involvement in disease: 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. Ref.2 Ref.28 Ref.35Defects in PLEC 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.Defects in PLEC 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.Defects in PLEC are the cause of limb-girdle muscular dystrophy type 2Q (LGMD2Q) [

MIM:613723]. An autosomal recessive degenerative myopathy characterized by early childhood onset of proximal muscle weakness. Note=A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin. Ref.2 Ref.26

Sequence similarities: 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.