protein

Recombinant Plakophilin 2 (PKP2)

MBS2029229

0.01 mg

145 USD

Recombinant Protein

Recombinant Plakophilin 2 (PKP2)

[Plakophilin 2]

[Plakophilin 2; Plakophilin-2; plakophilin-2; plakophilin 2]

[PKP2]

[PKP2; PKP2; ARVD9]

PKP2_HUMAN

E.coli

Human

>95%

100mM NaHCO3, 500mM NaCl, pH8.3, containing 1mM EDTA, 1mM DTT, 0.01% sarcosyl, 5%Trehalose and Proclin300.

200 ug/ml

Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. The loss of this protein is less than 5% within the expiration date under appropriate storage condition.

SDS-PAGE, WB, ELISA, IP, CoIP, Purification, Amine Reactive Labeling. (May be suitable for use in other assays to be determined by the end user.)

Sequence Information

SDS-Page

Source: Prokaryotic expression. Residues: Asp571~Ala849. Tags: N-terminal His-Tag. Tissue Specificity: Brain, Kidney. Subcellular Location: Nucleus. Cell junction, desmosome. Traits: Freeze-dried powder. Predicted isoelectric point: 8.7. Predicted Molecular Mass: 34.6kDa. Accurate Molecular Mass: 35kDa as determined by SDS-PAGE reducing conditions.

Usage: Reconstitute in 100mM NaHCO3, 500mM NaCl (pH8.3) to a concentration of 0.1-1.0 mg/mL. Do not vortex.

219518981

AAI43967.1

Arrhythmogenic Right Ventricular Cardiomyopathy Pathway (672454); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (117293); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (116129)

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 gene product may regulate the signaling activity of beta-catenin. Two alternately spliced transcripts encoding two protein isoforms have been identified. A processed pseudogene with high similarity to this locus has been mapped to chromosome 12p13. [provided by RefSeq, Jul 2008]

plakophilin 2: May play a role in junctional plaques. Defects in PKP2 are the cause of familial arrhythmogenic right ventricular dysplasia type 9 (ARVD9); also known as arrhythmogenic right ventricular cardiomyopathy 9 (ARVC9). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall. Belongs to the beta-catenin family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Motility/polarity/chemotaxis; Cell adhesion; Membrane protein, integral. Chromosomal Location of Human Ortholog: 12p11. Cellular Component: nucleoplasm; desmosome; adherens junction; integral to membrane; plasma membrane; intermediate filament; intercellular junction; nucleus; cell junction. Molecular Function: protein binding; sodium channel regulator activity; protein kinase C binding; intermediate filament binding; protein complex scaffold. Biological Process: negative regulation of cell proliferation; cell-cell adhesion; gap junction assembly; heart development; lipid homeostasis; ventricular cardiac muscle morphogenesis; intermediate filament bundle assembly; maintenance of organ identity; negative regulation of cell migration. Disease: Arrhythmogenic Right Ventricular Dysplasia, Familial, 9

0.01 mg

145 USD

0.05 mg

285

0.1 mg

445

0.2 mg

545

0.5 mg

1055

1 mg

1575