Guinea Pig Plakophilin 1 Polyclonal Antibody | anti-PKP1 antibody
Plakophilin 1 antibody
WB: 1:1000
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the arm-repeat (armadillo) and plakophilin gene families. Plakophilin proteins contain numerous armadillo repeats, localize to cell desmosomes and nuclei, and participate in linking cadherins to intermediate filaments in the cytoskeleton. This protein may be involved in molecular recruitment and stabilization during desmosome formation. Mutations in this gene have been associated with the ectodermal dysplasia/skin fragility syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2010]
Uniprot Description
plakophilin 1: Seems to play a role in junctional plaques. Contributes to epidermal morphogenesis. Defects in PKP1 are the cause of ectodermal dysplasia- skin fragility syndrome (EDSFS); also known as McGrath syndrome. Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. EDSFS is characterized by features of both cutaneous fragility and congenital ectodermal dysplasia affecting abnormalities in other epithelia or tissues. Desmosomes in the skin are small and poorly formed with widening of keratinocyte intercellular spaces and perturbed desmosome/keratin intermediate filament interactions. Belongs to the beta-catenin family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Motility/polarity/chemotaxis; Cell adhesion
Chromosomal Location of Human Ortholog: 1q32
Cellular Component: nucleoplasm; desmosome; intracellular membrane-bound organelle; plasma membrane; intermediate filament; nucleus
Molecular Function: signal transducer activity; protein binding; intermediate filament binding; structural constituent of epidermis; lamin binding
Biological Process: cell-cell adhesion; apoptosis; multicellular organismal development; intermediate filament bundle assembly; signal transduction; cell adhesion; cell structure disassembly during apoptosis
Disease: Ectodermal Dysplasia/skin Fragility Syndrome
Research Articles on PKP1
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Product Notes
The PKP1 pkp1 (Catalog #AAA834823) is an Antibody produced from Guinea Pig and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's Plakophilin 1 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), Western Blot (WB). IHC: 1:100 WB: 1:1000. Researchers should empirically determine the suitability of the PKP1 pkp1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Plakophilin 1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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