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PLEC Produkte

(Plectin (PLEC))

Kategorien

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 These names were superseded by plectin.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].

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Ausgewählte PLEC Kategorien

PLEC Antikörper

High quality antibodies with extensive validation data.

PLEC ELISA Kits

Reliable ELISA kits for a wide range of species.

Empfohlene PLEC Antikörper

Produkt
Reaktivität
Applikation
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität Human
Applikation ELISA, IHC, IF
Validierungen
  • (3)
Kat. Nr. ABIN7163687
Menge 100 μg
Datenblatt Datenblatt
Reaktivität Human
Applikation ELISA, WB, IF
Validierungen
  • (3)
Kat. Nr. ABIN518954
Menge 100 μg
Datenblatt Datenblatt
Reaktivität Human, Mouse, Rat
Applikation WB, IHC (p)
Validierungen
  • (4)
Kat. Nr. ABIN3043900
Menge 100 μg
Datenblatt Datenblatt

Empfohlene PLEC ELISA Kits

Produkt
Reaktivität
Analytical Method
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität Human
Analytical Method Quantitative Sandwich ELISA
Validierungen
  • (1)
Kat. Nr. ABIN6969427
Menge 96 tests
Datenblatt Datenblatt
Reaktivität Human
Analytical Method Quantitative Sandwich ELISA
Validierungen
  • (1)
Kat. Nr. ABIN6958656
Menge 96 tests
Datenblatt Datenblatt
Reaktivität Rat
Analytical Method Quantitative Sandwich ELISA
Validierungen
  • (1)
Kat. Nr. ABIN6958657
Menge 96 tests
Datenblatt Datenblatt

Empfohlene PLEC Proteine

Produkt
Reaktivität
Source
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität Human
Source Wheat germ
Validierungen
  • (1)
Kat. Nr. ABIN1315362
Menge 10 μg
Datenblatt Datenblatt
Reaktivität Human
Source Escherichia coli (E. coli)
Validierungen
Kat. Nr. ABIN7396745
Menge 100 μg
Datenblatt Datenblatt
Reaktivität Human
Source HEK-293 Cells
Validierungen
Kat. Nr. ABIN7447921
Menge 100 μg
Datenblatt Datenblatt

Neueste Publikationen zu unseren PLEC Produkten

Domke, Franke et al.: "The cell-cell junctions of mammalian testes: II. The lamellar smooth muscle monolayer cells of the peritubular wall are laterally connected by vertical adherens junctions-a novel architectonic ..." in: Cell and tissue research, Vol. 375, Issue 2, pp. 451-482, (2019) (PubMed).

Liu, Wu, Lin, Liu, Chen: "[Fusion expression of SLT-IIeB gene and FedF gene of Ee in Escherichia coli and its immunogenicity]" in: Wei sheng wu xue bao = Acta microbiologica Sinica, Vol. 47, Issue 6, pp. 1044-9, (2008) (PubMed).

Banwell, Russel, Fukudome, Shen, Stilling, Engel: "Myopathy, myasthenic syndrome, and epidermolysis bullosa simplex due to plectin deficiency." in: Journal of neuropathology and experimental neurology, Vol. 58, Issue 8, pp. 832-46, (1999) (PubMed).

Andrä, Nikolic, Stöcher, Drenckhahn, Wiche: "Not just scaffolding: plectin regulates actin dynamics in cultured cells." in: Genes & development, Vol. 12, Issue 21, pp. 3442-51, (1998) (PubMed).

Liu, Maercker, Castañon, Hauptmann, Wiche: "Human plectin: organization of the gene, sequence analysis, and chromosome localization (8q24)." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 93, Issue 9, pp. 4278-83, (1996) (PubMed).

Wiche, Krepler, Artlieb, Pytela, Denk: "Occurrence and immunolocalization of plectin in tissues." in: The Journal of cell biology, Vol. 97, Issue 3, pp. 887-901, (1983) (PubMed).

Synonyme und alternative Namen zu PLEC

plectin (LOC100481602), plectin L homeolog (plec.L), plectin (PLEC), plectin (Plec), plectin (LOC786966), AA591047, AU042537, EBS1, EBSO, HD1, IFAP300, LGMD2Q, PCN, Pcn, PLEC1, Plec1, PLEC1b, Plectin-1, PLTN, Pltn

Bezeichner auf Proteinebene für PLEC

  • plectin
  • plectin-1-like
  • hemidesmosomal protein 1
  • plectin 1, intermediate filament binding protein 500kDa
  • plectin-1
  • plectin 1
  • plectin-6
  • 300 kDa intermediate filament-associated protein
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