Plakophilin 1, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against PKP1. It has beenselected for its ability to recognize PKP1 in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-PKP1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.2-2ug/mL;1:250-2500
Immunohistochemistry: 5-20ug/mL;1:25-100
Immunocytochemistry: 5-20ug/mL;1:25-100
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded kidney tissue)

DAB staining on IHC-P; Samples: Human Kidney Tissue)

80,497 Da

plakophilin 1

plakophilin-1

Plakophilin-1

B6P  [Similar Products]

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]

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: Cell adhesion; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 1q32.1

Cellular Component: cornified envelope; desmosome; extracellular exosome; intermediate filament; intracellular membrane-bound organelle; nucleoplasm; nucleus; plasma membrane

Molecular Function: intermediate filament binding; lamin binding; protein binding; signal transducer activity; structural constituent of epidermis

Biological Process: cell adhesion; cornification; intermediate filament bundle assembly; keratinization; negative regulation of mRNA catabolic process; neutrophil degranulation; positive regulation of gene expression; signal transduction

Disease: Ectodermal Dysplasia/skin Fragility Syndrome

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