Anti-Plectin Antibody (CAB19743)

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SKU:
CAB19743
Product Type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
EBS1
Synonyms:
EBSMD
Synonyms:
EBSND
Synonyms:
EBSO
Synonyms:
EBSOG
Synonyms:
EBSPA
Synonyms:
HD1
Synonyms:
LGMD2Q
Synonyms:
PCN
Synonyms:
PLEC1
Synonyms:
PLEC1b
Synonyms:
PLTN

Description

Product Name:Plectin Rabbit mAb
Product Code:CAB19743
Size:20uL, 50uL, 100uL
Synonyms:EBS1, EBSMD, EBSND, EBSO, EBSOG, EBSPA, HD1, LGMD2Q, PCN, PLEC1, PLEC1b, PLTN
Applications:IHC
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human Plectin
Applications:IHC
Recommended Dilutions:IHC 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Immunogen:A synthesized peptide derived from human Plectin
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:5339
Uniprot:Q15149
Calculated MW:531kDa
UniProt Protein Function:Plectin-1: a major cytoskeleton crosslinking protein that binds to actin, intermediate filaments and microtubules. A scaffold for proteins involved in cellular signaling. 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. Regulates the organization of keratin intermediate filaments (IF) and MAPK signaling during cell migration. Downregulates PKC signaling by sequestering RACK1. Redistributes to the uropod associated with vimentin and fodrin during polarization of peripheral blood T lymphocytes. This vimentin-plectin-fodrin complex provides a continuous linkage from the nucleus (lamin B) to the cortical cytoskeleton. Defects in PLEC1 are the cause of epidermolysis bullosa simplex 1 (EBS1). Three alternatively spliced isoforms have been described.
UniProt Protein Details:

Protein type:Cytoskeletal; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 8q24

Cellular Component: intermediate filament cytoskeleton; costamere; focal adhesion; hemidesmosome; cytoplasm; plasma membrane; cytosol; sarcoplasm; sarcolemma

Molecular Function:protein binding; structural constituent of muscle; ankyrin binding; actin binding

Biological Process: extracellular matrix organization and biogenesis; hemidesmosome assembly; apoptosis; cell structure disassembly during apoptosis

NCBI Summary: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 "plectin 1, intermediate filament binding 500kDa". These names were 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). 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. [provided by RefSeq, Aug 2011]
UniProt Code:Q15149
NCBI GenInfo Identifier:209572726
NCBI Gene ID:5339
NCBI Accession:Q15149.3
UniProt Secondary Accession:Q15149,Q15148, Q16640, Q6S376, Q6S377, Q6S378, Q6S379 Q6S380, Q6S381, Q6S382, Q6S383,
UniProt Related Accession:Q15149
Molecular Weight:514,775 Da
NCBI Full Name:Plectin
NCBI Synonym Full Names:plectin
NCBI Official Symbol:PLEC  
NCBI Official Synonym Symbols:HD1; PCN; EBS1; EBSO; PLTN; PLEC1; LGMD2Q; PLEC1b  
NCBI Protein Information:plectin; hemidesmosomal protein 1; plectin 1, intermediate filament binding protein 500kDa
UniProt Protein Name:Plectin
UniProt Synonym Protein Names:Hemidesmosomal protein 1; HD1; Plectin-1
Protein Family:Plectin
UniProt Gene Name:PLEC  
UniProt Entry Name:PLEC_HUMAN
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