catalog number :
MBS531465
products full name :
Plakophilin 1 antibody
products short name :
[Plakophilin 1]
products name syn :
[Monoclonal Plakophilin 1; Anti-Plakophilin 1]
other names :
[plakophilin 1; Plakophilin-1; plakophilin-1; plakophilin 1; Band 6 protein; B6P]
other gene names :
[PKP1; PKP1; B6P; B6P]
uniprot entry name :
PKP1_HUMAN
reactivity :
Human, cow, pig, Xenopus laevis. Reacts on cultured lines HaCat, and A-431.
specificity :
Bovine plakophilin 1
purity :
Culture supernatant
form :
Cell culture supernatant
storage stability :
Store at 4°C.
tested application :
Suitable for use in Western Blot, Immunohistochemistry on cryostat and paraffin-embedded tissue sections (P)- requires pretreatment with 0.001% trypsin together with microwave antigen release, and ELISA.
other info1 :
Biological Significance: Plakophilin-1 is a protein that in humans is encoded by the PKP1 gene. 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.
other info2 :
Immunogen: Plakophilin 1("band 6 polypeptide" of Bovine Muzzle epidermis).
products categories :
Cell Biology
products description :
Mouse monoclonal Plakophilin 1 antibody
ncbi acc num :
CAA98022.1
ncbi pathways :
Apoptosis Pathway (105648); Apoptotic Cleavage Of Cell Adhesion Proteins Pathway (105680); Apoptotic Cleavage Of Cellular Proteins Pathway (105678); Apoptotic Execution Phase Pathway (105677); Programmed Cell Death Pathway (1127510)
ncbi summary :
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 summary :
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: 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