Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Plakophilin-1 (PKP1) Standard Curve.)
Human Plakophilin-1 (PKP1) ELISA Kit | PKP1 elisa kit
Human Plakophilin-1 (PKP1) ELISA Kit
* Allow all reagents to warm to room temperature for at least 30 minutes before opening.
* Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.
* Unused wells must be kept desiccated at 4 degree C in the sealed bag provided.
* Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.
* It is recommended that all samples and standards be assayed in duplicate or triplicate.
Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Plakophilin-1 (PKP1) Standard Curve.)
Typical Testing Data/Standard Curve (for reference only)
(Fig.1. Human Plakophilin-1 (PKP1) Standard Curve.)
Background: Plakophilin-1 is 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.Compared with those in controls, desmosomes in PKP1-null patients were reduced dramatically both in size (49%) and frequency (61%) in the lower suprabasal layers (P less than 0.01). In the lower suprabasal compartment of the heterozygous carrier, corresponding reductions were 37% and 20%, respectively (P less than 0.01).
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: Cell adhesion; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 1q32
Cellular Component: nucleoplasm; desmosome; intracellular membrane-bound organelle; plasma membrane; intermediate filament; nucleus
Molecular Function: protein binding; signal transducer activity; intermediate filament binding; structural constituent of epidermis; lamin binding
Biological Process: cell-cell adhesion; apoptosis; multicellular organismal development; intermediate filament bundle assembly; cell adhesion; signal transduction; cell structure disassembly during apoptosis
Disease: Ectodermal Dysplasia/skin Fragility Syndrome
Research Articles on PKP1
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Product Notes
The Human PKP1 pkp1 (Catalog #AAA9715872) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9715872 ELISA Kit recognizes Human PKP1. It is sometimes possible for the material contained within the vial of "Plakophilin-1 (PKP1), ELISA Kit" 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
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